Displaying publications 41 - 60 of 266 in total

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  1. Oyehan TA, Alade IO, Bagudu A, Sulaiman KO, Olatunji SO, Saleh TA
    Comput Biol Med, 2018 07 01;98:85-92.
    PMID: 29777986 DOI: 10.1016/j.compbiomed.2018.04.024
    The optical properties of blood play crucial roles in medical diagnostics and treatment, and in the design of new medical devices. Haemoglobin is a vital constituent of the blood whose optical properties affect all of the optical properties of human blood. The refractive index of haemoglobin has been reported to strongly depend on its concentration which is a function of the physiology of biological cells. This makes the refractive index of haemoglobin an essential non-invasive bio-marker of diseases. Unfortunately, the complexity of blood tissue makes it challenging to experimentally measure the refractive index of haemoglobin. While a few studies have reported on the refractive index of haemoglobin, there is no solid consensus with the data obtained due to different measuring instruments and the conditions of the experiments. Moreover, obtaining the refractive index via an experimental approach is quite laborious. In this work, an accurate, fast and relatively convenient strategy to estimate the refractive index of haemoglobin is reported. Thus, the GA-SVR model is presented for the prediction of the refractive index of haemoglobin using wavelength, temperature, and the concentration of haemoglobin as descriptors. The model developed is characterised by an excellent accuracy and very low error estimates. The correlation coefficients obtained in these studies are 99.94% and 99.91% for the training and testing results, respectively. In addition, the result shows an almost perfect match with the experimental data and also demonstrates significant improvement over a recent mathematical model available in the literature. The GA-SVR model predictions also give insights into the influence of concentration, wavelength, and temperature on the RI measurement values. The model outcome can be used not only to accurately estimate the refractive index of haemoglobin but also could provide a reliable common ground to benchmark the experimental refractive index results.
    Matched MeSH terms: Hemoglobins/analysis*
  2. Alade IO, Bagudu A, Oyehan TA, Rahman MAA, Saleh TA, Olatunji SO
    Comput Methods Programs Biomed, 2018 Sep;163:135-142.
    PMID: 30119848 DOI: 10.1016/j.cmpb.2018.05.029
    BACKGROUND AND OBJECTIVES: The refractive index of hemoglobin plays important role in hematology due to its strong correlation with the pathophysiology of different diseases. Measurement of the real part of the refractive index remains a challenge due to strong absorption of the hemoglobin especially at relevant high physiological concentrations. So far, only a few studies on direct measurement of refractive index have been reported and there are no firm agreements on the reported values of refractive index of hemoglobin due to measurement artifacts. In addition, it is time consuming, laborious and expensive to perform several experiments to obtain the refractive index of hemoglobin. In this work, we proposed a very rapid and accurate computational intelligent approach using Genetic Algorithm/Support Vector Regression models to estimate the real part of the refractive index for oxygenated and deoxygenated hemoglobin samples.

    METHODS: These models utilized experimental data of wavelengths and hemoglobin concentrations in building highly accurate Genetic Algorithm/Support Vector Regression model (GA-SVR).

    RESULTS: The developed methodology showed high accuracy as indicated by the low root mean square error values of 4.65 × 10-4 and 4.62 × 10-4 for oxygenated and deoxygenated hemoglobin, respectively. In addition, the models exhibited 99.85 and 99.84% correlation coefficients (r) for the oxygenated and deoxygenated hemoglobin, thus, validating the strong agreement between the predicted and the experimental results CONCLUSIONS: Due to the accuracy and relative simplicity of the proposed models, we envisage that these models would serve as important references for future studies on optical properties of blood.

    Matched MeSH terms: Hemoglobins/chemistry*
  3. Muthukumaravel K, Priyadharshini M, Kanagavalli V, Vasanthi N, Ahmed MS, Musthafa MS, et al.
    Environ Monit Assess, 2022 Oct 21;195(1):10.
    PMID: 36269455 DOI: 10.1007/s10661-022-10554-2
    Phenol, an aromatic chemical commonly found in domestic and industrial effluents, upon its introduction into aquatic ecosystems adversely affects the indigenous biota, the invertebrates and the vertebrates. With the increased demand for agrochemicals, a large amount of phenol is released directly into the environment as a byproduct. Phenol and its derivatives tend to persist in the environment for longer periods which in turn poses a threat to both humans and the aquatic ecosystem. In our current study, the response of Labeo rohita to sublethal concentrations of phenol was observed and the results did show a regular decrease in biochemical constituents of the targeted organs. Exposure of Labeo rohita to sublethal concentration of phenol (22.32 mg/L) for an epoch of 7, 21 and 28 days shows a decline in lipid, protein, carbohydrate content and phosphatase activity in target organs such as the gills, muscle, intestine, liver and kidney of the fish. The present study also aims to investigate the toxic effects of phenol with special reference to the haematological parameters of Labeo rohita. At the end of the exposure period, the blood of the fish was collected by cutting the caudal peduncle with a surgical scalpel. And it was observed that the red blood corpuscle count (RBC), white blood corpuscle (WBC), haemoglobin count (Hb), packed cell volume (PCV), mean corpuscular volume (MCV), mean corpuscular haemoglobin (MCH) and mean corpuscular haemoglobin concentration (MCHC) values showed a decline after exposure to phenol for 7 days, while white blood corpuscle (WBC) shows an increased count. At 21 days and 28 days, all the haematological parameters showed a significant decrease.
    Matched MeSH terms: Hemoglobins/metabolism
  4. Amran HS, Aziz MA, George E, Mahmud N, Lee TY, Md Noor S
    Malays J Pathol, 2017 Dec;39(3):321-326.
    PMID: 29279598 MyJurnal
    Hb Tak is one of more than 200 high affinity haemoglobin variants reported worldwide. It results from the insertion of two nucleotides (AC) at the termination codon, between codon 146 and codon 147 of the beta-globin gene [Beta 147 (+AC)]. Polycythaemia is the main clinical feature although affected carriers are usually asymptomatic and do not require intervention. Several case studies in this region have reported the co-inheritance of Hb Tak with Hb E, delta beta and beta thalassaemia with one case of homozygous Hb Tak in a Thai boy. In this case report, a cluster of haemoglobin Tak was found in a family of Malay ethnic origin. Cascade family screening was conducted while investigating a 4-year old girl who presented with symptomatic polycythaemia. She had 2 previous Hb analysis done, at 7-month and 2-year-old with the diagnosis of possible Hb Q Thailand and Homozygous Hb D, respectively. Both diagnosis did not fit her clinical presentations. She was plethoric, had reduced exercise tolerance as well as cardiomyopathy. Her parents were consanguineously married and later diagnosed as asymptomatic carriers of Hb Tak. Consequently, re-analysis of the girl's blood sample revealed a homozygous state of Hb Tak. In conclusion, high oxygen affinity haemoglobin like Hb Tak should be considered in the investigation of polycythaemic patients with abnormal Hb analyses. In this case, DNA analysis was crucial in determining the correct diagnosis.
    Matched MeSH terms: Hemoglobins, Abnormal/genetics*
  5. Cooper DJ, Grigg MJ, Plewes K, Rajahram GS, Piera KA, William T, et al.
    Clin Infect Dis, 2022 Oct 12;75(8):1379-1388.
    PMID: 35180298 DOI: 10.1093/cid/ciac152
    BACKGROUND: Acetaminophen inhibits cell-free hemoglobin-induced lipid peroxidation and improves renal function in severe falciparum malaria but has not been evaluated in other infections with prominent hemolysis, including Plasmodium knowlesi malaria.

    METHODS: PACKNOW was an open-label, randomized, controlled trial of acetaminophen (500 mg or 1000 mg every 6 hours for 72 hours) vs no acetaminophen in Malaysian patients aged ≥5 years with knowlesi malaria of any severity. The primary end point was change in creatinine at 72 hours. Secondary end points included longitudinal changes in creatinine in patients with severe malaria or acute kidney injury (AKI), stratified by hemolysis.

    RESULTS: During 2016-2018, 396 patients (aged 12-96 years) were randomized to acetaminophen (n = 199) or no acetaminophen (n = 197). Overall, creatinine fell by a mean (standard deviation) 14.9% (18.1) in the acetaminophen arm vs 14.6% (16.0) in the control arm (P = .81). In severe disease, creatinine fell by 31.0% (26.5) in the acetaminophen arm vs 20.4% (21.5) in the control arm (P = .12), and in those with hemolysis by 35.8% (26.7) and 19% (16.6), respectively (P = .07). No difference was seen overall in patients with AKI; however, in those with AKI and hemolysis, creatinine fell by 34.5% (20.7) in the acetaminophen arm vs 25.9% (15.8) in the control arm (P = .041). Mixed-effects modeling demonstrated a benefit of acetaminophen at 72 hours (P = .041) and 1 week (P = .002) in patients with severe malaria and with AKI and hemolysis (P = .027 and P = .002, respectively).

    CONCLUSIONS: Acetaminophen did not improve creatinine among the entire cohort but may improve renal function in patients with severe knowlesi malaria and in those with AKI and hemolysis.

    CLINICAL TRIALS REGISTRATION: NCT03056391.

    Matched MeSH terms: Hemoglobins/therapeutic use
  6. Yong HY, Mohd Shariff Z, Mohd Yusof BN, Rejali Z, Tee YYS, Bindels J, et al.
    Br J Nutr, 2022 Dec 14;128(11):2097-2104.
    PMID: 35139935 DOI: 10.1017/S000711452100502X
    This study aimed to determine the association between hemoglobin (Hb) concentration and Hb change, during early to mid-pregnancy with the risk of gestational diabetes mellitus (GDM). This was a clinic-based retrospective cohort study of 1951 healthy pregnant women (18-45 years old) with a singleton gestation attending antenatal care at government health clinics. Hb concentration at first prenatal visit and each trimester was extracted from the antenatal cards. Hb changes from first prenatal visit to first and second trimester as well as from second to third trimester were calculated. Multivariate logistic regression was used with adjustment for covariates. Women with GDM had significantly higher Hb concentrations (Hb 1) at first prenatal visit (< 12 weeks) compared with non-GDM women (11·91 g/dl v.11·74 g/dl). Hb 1 and Hb changes (Hb change 2) from first prenatal visit to the second trimester (23-27th weeks) were significantly associated with GDM risk, with an adjusted OR of 1·14 (95 % CI 1·01, 1·29) and 1·25 (95 % CI 1·05, 1·49), respectively. The significant associations between Hb 1 and Hb change 2 with the risk of GDM were found among non-Malays, overweight/obese and women aged 35 years and above. Women with higher Hb concentrations in early pregnancy were at higher risk of GDM, and such association was significant among women aged 35 years and above, non-Malays and overweight/obese. This raises a potential concern for elevated Fe status in early pregnancy as a risk factor of GDM among Fe-replete women.
    Matched MeSH terms: Hemoglobins/analysis
  7. Hassan K, Vijayasilan T, Mahmood Z, Abdul Hamid H, Chin YM
    Singapore Med J, 1988 Oct;29(5):462-8.
    PMID: 3241975
    Whole blood samples from patients with various forms of alpha- and beta- thalassaemia were incubated with 14C-Leucine to determine the relative rates of production of the alpha and beta chains by their reticulocytes. The labelled globin chains were fractionated by CM-Cellulose Chromatography in 8M Urea and the incorporated activity determined. The relative rates of synthesis of alpha and beta chains in some cases of alpha and beta- thalassaemia were established and the chain synthetic ratios were compared with similar ratios in normal individuals. The results show that it is possible to identify from the relative rates of in-vitro synthesis of the alpha and beta chains, the presence of the common thalassaemia slates in particular beta-thal trait, beta-thal homozygotes, Hb H disease and alpha0-thal trait. The presence of transfused blood does not affect the result. This study indicates that an abnormal alpha/beta chain synthesis ratio is useful in defining alpha and beta-thalassaemia variants.
    Matched MeSH terms: Hemoglobins/biosynthesis*
  8. Musallam KM, Lombard L, Kistler KD, Arregui M, Gilroy KS, Chamberlain C, et al.
    Am J Hematol, 2023 Sep;98(9):1436-1451.
    PMID: 37357829 DOI: 10.1002/ajh.27006
    This systematic literature review assessed the global prevalence and birth prevalence of clinically significant forms of alpha- and beta-thalassemia. Embase, MEDLINE, and the Cochrane Library were searched for observational studies published January 1, 2000, to September 21, 2021. Of 2093 unique records identified, 69 studies reported across 70 publications met eligibility criteria, including 6 records identified from bibliography searches. Thalassemia prevalence estimates varied across countries and even within countries. Across 23 population-based studies reporting clinically significant alpha-thalassemia (e.g., hemoglobin H disease and hemoglobin Bart's hydrops fetalis) and/or beta-thalassemia (beta-thalassemia intermedia, major, and/or hemoglobin E/beta-thalassemia), prevalence estimates per 100 000 people ranged from 0.2 in Spain (over 2014-2017) to 27.2 in Greece (2010-2015) for combined beta- plus alpha-thalassemia; from 0.03 in Spain (2014-2017) to 4.5 in Malaysia (2007-2018) for alpha-thalassemia; and from 0.2 in Spain (2014-2017) to 35.7 to 49.6 in Iraq (2003-2018) for beta-thalassemia. Overall, the estimated prevalence of thalassemia followed the predicted pattern of being higher in the Middle East, Asia, and Mediterranean than in Europe or North America. However, population-based prevalence estimates were not found for many countries, and there was heterogeneity in case definitions, diagnostic methodology, type of thalassemia reported, and details on transfusion requirements. Limited population-based birth prevalence data were found. Twenty-seven studies reported thalassemia prevalence from non-population-based samples. Results from such studies likely do not have countrywide generalizability as they tended to be from highly specific groups. To fully understand the global prevalence of thalassemia, up-to-date, population-based epidemiological data are needed for many countries.
    Matched MeSH terms: Hemoglobins, Abnormal*
  9. Lie-Injo LE, Randhawa ZI, Ganesan J, Kane J, Peterson D
    Hemoglobin, 1977;1(8):747-57.
    PMID: 604313
    The trait condition for hemoglobin Tak, was found in a 4-day old newborn Malay who suffered from severe neonatal jaundice. The beta chain of the abnormal hemoglobin was elongated by 11 residues at the C-terminus and had the same structure as reported for Hb Tak. The mother was heterozygous for this abnormal hemoglobin, the father was normal. The mother and child, 4 years later, did not show clinical or hematological symptoms except definitely increased resistance of their erythrocytes to hypotonic saline solutions and slight anisopoikilocytosis. The abnormal gene in the two reported Thai families and in our Malay family may have the same origin.
    Matched MeSH terms: Hemoglobins, Abnormal/isolation & purification*
  10. Fix AG, Lie-injo LE
    Am J Phys Anthropol, 1975 Jul;43(1):47-55.
    PMID: 1155591
    Blood samples, demographic and cultural data were collected from seven settlements of Semai Senoi, a swidden farming ethnic group of Malaysia. Three genetic loci (ABO blood group, hereditary ovalcytosis, and hemoglobin) were analyzed in a total sample of 546 individuals. These data indicate a considerable degree of genetic microdifferentiation in this area of the Semai distribution. Parent-offspring birthplace data (analyzed by means of a migration matrix) and settlement histories show that settlements are not strongly isolated. Genetic differences in the study area demonstrate a reasonable correspondence with migration and the history of the settlements. Genetic convergence also occurs through the addition of migrant groups to established populations leading to new patterns of marriage between donor and recipient groups. The genetic structure of the total Semai population through time thus comprises a mosaic of shifiting allele frequencies in a series of semi-isolated local populations.
    Matched MeSH terms: Hemoglobins/analysis
  11. Nute PE, Pataryas HA
    Am J Phys Anthropol, 1974 Jan;40(1):17-25.
    PMID: 4206325
    Matched MeSH terms: Hemoglobins/analysis*
  12. Ton SH, Lopez CG
    PMID: 7403958
    Serum ferritin and haemoglobin estimates were carried out on 78 first time blood donors with a view to determining iron store status. Of these 30 were Malays, 20 were Chinese and 28 were Indians. The ferritin level in Malay donors ranged from 16-160 mg/ml (mean 83 +/- 49.4 mg/ml in chinese donors is ranged from 36-500 mg/ml (mean 242.8 +/- 132 mg/ml), and in the Indian donors it ranged from 5 - 270 mg/ml (mean 94.6 +/- 67.9 mg/ml). The haemoglobin concentration for the whole group was 14.9 +/- 1.49 g/dl. There was no correlation of haemoglobin concentration with serum ferritin levels.
    Matched MeSH terms: Hemoglobins/analysis
  13. Raja Jamaluddin RZA, Yook Heng L, Tan LL, Chong KF
    Sensors (Basel), 2018 Apr 26;18(5).
    PMID: 29701688 DOI: 10.3390/s18051343
    A new biosensor for the analysis of nitrite in food was developed based on hemoglobin (Hb) covalently immobilized on the succinimide functionalized poly(n-butyl acrylate)-graphene [poly(nBA)-rGO] composite film deposited on a carbon-paste screen-printed electrode (SPE). The immobilized Hb on the poly(nBA)-rGO conducting matrix exhibited electrocatalytic ability for the reduction of nitrite with significant enhancement in the reduction peak at −0.6 V versus Ag/AgCl reference electrode. Thus, direct determination of nitrite can be achieved by monitoring the cathodic peak current signal of the proposed polyacrylic-graphene hybrid film-based voltammetric nitrite biosensor. The nitrite biosensor exhibited a reproducible dynamic linear response range from 0.05⁻5 mg L−1 nitrite and a detection limit of 0.03 mg L−1. No significant interference was observed by potential interfering ions such as Ca2+, Na⁺, K⁺, NH₄⁺, Mg2+, and NO₃− ions. Analysis of nitrite in both raw and processed edible bird’s nest (EBN) samples demonstrated recovery of close to 100%. The covalent immobilization of Hb on poly(nBA)-rGO composite film has improved the performance of the electrochemical nitrite biosensor in terms of broader detection range, lower detection limit, and prolonged biosensor stability.
    Matched MeSH terms: Hemoglobins
  14. Mohamad AS, Hamzah R, Selvaratnam V, Yegapan S, Sathar J
    Hematol Rep, 2018 Sep 05;10(3):7210.
    PMID: 30344984 DOI: 10.4081/hr.2018.7210
    Human hemoglobin of G-Makassar variant has been reported very rarely with Beta Thalassemia. In year 1969 Hb GMakassar was first identified in Makassar, Sulawesi (Celebes), Republic of Indonesia. The disease was first published in 1969 and 33 years later it has been reported at a family of Thailand origin. We report a 45-yearold Malay man who was investigated for anemia and thrombocytopenia then diagnosed with Hb G-Makassar. This finding describes as a new Hemoglobin GMakassar discovered in Malaysia after 14 years diagnosed in Thailand.
    Matched MeSH terms: Hemoglobins, Abnormal
  15. Abd Rahman R, Idris IB, Md Isa Z, Abd Rahman R
    PLoS One, 2022;17(12):e0278192.
    PMID: 36473006 DOI: 10.1371/journal.pone.0278192
    Anemia in pregnancy is a public health concern. It has been diagnosed in 27% of pregnant women in Malaysia and up to 40% of pregnant women globally. This study aimed to develop and evaluate the effectiveness of an intervention initiative based on the health belief model. The MyPinkMom program was disseminated through a mobile messaging application to pregnant women to educate them on the prevention of anemia in pregnancy. We conducted a two-arm cluster-assignment, single-blinded, randomized control trial at two government antenatal clinics in Selangor. One clinic was randomly chosen as the intervention group, and the other was chosen as the control group. Sixty pregnant women with anemia from the intervention group received the MyPinkMom intervention program in the form of six infographic video clips, and 60 pregnant women with anemia from the control group received routine counseling on anemia in pregnancy. Pregnant women who had anemia secondary to hemoglobinopathy or other chronic diseases were excluded from this study. MANOVA showed significant increases in hemoglobin, knowledge, attitude, subjective norms, and perceived behavioral control scores for adherence to iron supplements, dietary iron, and dietary vitamin C intake (p < 0.001) in the intervention group at week 6. A significant reduction also occurred in dietary tannin intake (p < 0.001) in the intervention group at week 6. The intervention group at week 6 showed a large effect on hemoglobin level increments (partial eta squared, Ƞp2 0.268), dietary iron intake (Ƞp2 0.213), knowledge of anemia in pregnancy (Ƞp2 0.622), subjective norm scores for adherence to iron supplements (Ƞp2 0.167), and reduction in dietary tannin intake (Ƞp2 0.353). Similarly, repeated measures ANOVA showed that changes in hemoglobin levels were significantly different over time (i.e., at baseline, week 6, and week 12) between the intervention and control groups (p < 0.001). Hemoglobin increased rapidly over time among participants in the intervention group but gradually in the control group. To conclude, the newly developed MyPinkMom program that was delivered through a messaging application showed effectiveness in preventing anemia during pregnancy.
    Matched MeSH terms: Hemoglobins
  16. Kishi K, Jarutach J, Ooi YK, Wong NL, Che Mood M, Kandhavello G, et al.
    Cardiol Young, 2023 Aug;33(8):1359-1366.
    PMID: 35950412 DOI: 10.1017/S104795112200244X
    INTRODUCTION: Current guidelines discourage shunt closure in patients with pulmonary vascular resistance index >8 Wood units x m2. The study examined the long-term outcome of patients over 15 years old, with pulmonary vascular resistance index >8 Wood units x m2 and patent ductus arteriosus.

    MATERIALS AND METHODS: This was a multi-institutional, retrospective study involving all consecutive patients (>15 years old) with patent ductus arteriosus and severe pulmonary hypertension. Patients who had patent ductus arteriosus closure were divided into the Good (no death or hospital admissions due to worsening pulmonary hypertension) and the Poor Outcome groups and these groups were compared.

    RESULTS: Thirty-seven patients [male: 9 (24.3%); mean age: 30.49 ± 9.56 years; median follow-up: 3 (IQR: 1.5,10) years] were included from four centers. Twenty-two patients who underwent patent ductus arteriosus closure, 15 (71.4%) had good outcomes while 7 (28.6%) had poor outcomes. Pulmonary vascular resistance index and pulmonary to systemic resistance ratio (Rp:Rs) were lower in the Good Outcome Group (14.35 ± 1.66 Wood units x m2 vs. 20.07 ± 2.44; p = 0.033 and 0.44 ± 0.16 vs. 1.08 ± 1.21; p = 0.042). Haemoglobin concentrations (<14.3 g/dL) were associated with good long-term outcomes in the Closed Group.

    CONCLUSIONS: Patients with patent ductus arteriosus with severe pulmonary hypertension have a dismal outcome with or without closure. High haemoglobin levels at the time of occlusion predict a worse outcome for patients with patent ductus arteriosus and pulmonary hypertension.

    Matched MeSH terms: Hemoglobins
  17. Lee JW, Griffin M, Kim JS, Lee Lee LW, Piatek C, Nishimura JI, et al.
    Lancet Haematol, 2023 Dec;10(12):e955-e965.
    PMID: 38030318 DOI: 10.1016/S2352-3026(23)00315-0
    BACKGROUND: Symptoms of anaemia due to clinically significant extravascular haemolysis can affect patients with paroxysmal nocturnal haemoglobinuria (PNH) treated with C5 inhibitors (ravulizumab or eculizumab). The aim of this study was to assess the efficacy and safety of danicopan (ALXN2040), an investigational, first-in-class, oral complement factor D inhibitor, as add-on therapy to ravulizumab or eculizumab in patients with PNH and clinically significant extravascular haemolysis.

    METHODS: ALPHA is an ongoing, international, phase 3, randomised, double-blind, placebo-controlled trial evaluating danicopan as add-on therapy to ravulizumab or eculizumab. Eligible patients were adults (age ≥18 years) with PNH and clinically significant extravascular haemolysis (haemoglobin ≤9·5 g/dL; absolute reticulocyte count ≥120 × 109/L) on ravulizumab or eculizumab for at least 6 months. Patients were randomly assigned (2:1) to danicopan or placebo added to ravulizumab or eculizumab for 12 weeks using an interactive response technology system. Randomisation was stratified based on transfusion history, haemoglobin, and patients enrolled from Japan. The initial oral danicopan dose was 150 mg three times a day; escalation to 200 mg three times a day was permitted based on clinical response. The infusion dose level of eculizumab (every 2 weeks) ranged from 900 mg to 1500 mg, and for ravulizumab (monthly or every 8 weeks) ranged from 3000 mg to 3600 mg. The primary endpoint was change in haemoglobin concentration from baseline to week 12. Here we present the protocol-prespecified interim analysis, planned when approximately 75% of participants were randomly assigned to treatment and completed or discontinued at 12 weeks. This trial is registered with ClinicalTrials.gov (NCT04469465).

    FINDINGS: Individuals were randomly assigned between Dec 16, 2020, and Aug 29, 2022. At data cutoff (June 28, 2022), 73 individuals were randomly assigned, received treatment, and were analysed for safety (danicopan, n=49; placebo, n=24). The protocol-prespecified interim efficacy analysis set included the first 63 participants (danicopan, n=42; placebo, n=21). At week 12, danicopan plus ravulizumab or eculizumab increased haemoglobin versus placebo plus ravulizumab or eculizumab (least squares mean [LSM] change from baseline: danicopan, 2·94 g/dL [95% CI 2·52 to 3·36]; placebo, 0·50 g/dL [-0·13 to 1·12]; LSM difference, 2·44 g/dL [1·69 to 3·20]; p<0·0001). Grade 3 adverse events in the danicopan group were increased alanine aminotransferase (two [4%] of 49 patients), leukopenia (one [2%]), neutropenia (two [4%]), cholecystitis (one [2%]), COVID-19 (one [2%]), increased aspartate aminotransferase (one [2%]), and increased blood pressure (one [2%]), and in the placebo group were anaemia (one [4%] of 24 patients), thrombocytopenia (one [4%]), and asthenia (one [4%]). The serious adverse events reported in the danicopan group were cholecystitis (one [2%] patient) and COVID-19 (one [2%]) and in the placebo group were anaemia and abdominal pain, both in one (4%) patient. There were no serious adverse events related to study drug or deaths reported in the study.

    INTERPRETATION: These primary efficacy and safety results show that danicopan as add-on treatment to ravulizumab or eculizumab significantly improved haemoglobin concentrations at week 12 with no new safety concerns, suggesting an improved benefit-risk profile in patients with PNH and clinically significant extravascular haemolysis.

    FUNDING: Alexion, AstraZeneca Rare Disease.

    Matched MeSH terms: Hemoglobins
  18. Rajasekhar M, Simpson JA, Ley B, Edler P, Chu CS, Abreha T, et al.
    Lancet Infect Dis, 2024 Feb;24(2):184-195.
    PMID: 37748497 DOI: 10.1016/S1473-3099(23)00431-0
    BACKGROUND: Primaquine radical cure is used to treat dormant liver-stage parasites and prevent relapsing Plasmodium vivax malaria but is limited by concerns of haemolysis. We undertook a systematic review and individual patient data meta-analysis to investigate the haematological safety of different primaquine regimens for P vivax radical cure.

    METHODS: For this systematic review and individual patient data meta-analysis, we searched MEDLINE, Web of Science, Embase, and Cochrane Central for prospective clinical studies of uncomplicated P vivax from endemic countries published between Jan 1, 2000, and June 8, 2023. We included studies if they had active follow-up of at least 28 days, if they included a treatment group with daily primaquine given over multiple days where primaquine was commenced within 3 days of schizontocidal treatment and was given alone or coadministered with chloroquine or one of four artemisinin-based combination therapies (ie, artemether-lumefantrine, artesunate-mefloquine, artesunate-amodiaquine, or dihydroartemisinin-piperaquine), and if they recorded haemoglobin or haematocrit concentrations on day 0. We excluded studies if they were on prevention, prophylaxis, or patients with severe malaria, or if data were extracted retrospectively from medical records outside of a planned trial. For the meta-analysis, we contacted the investigators of eligible trials to request individual patient data and we then pooled data that were made available by Aug 23, 2021. The main outcome was haemoglobin reduction of more than 25% to a concentration of less than 7 g/dL by day 14. Haemoglobin concentration changes between day 0 and days 2-3 and between day 0 and days 5-7 were assessed by mixed-effects linear regression for patients with glucose-6-phosphate dehydrogenase (G6PD) activity of (1) 30% or higher and (2) between 30% and less than 70%. The study was registered with PROSPERO, CRD42019154470 and CRD42022303680.

    FINDINGS: Of 226 identified studies, 18 studies with patient-level data from 5462 patients from 15 countries were included in the analysis. A haemoglobin reduction of more than 25% to a concentration of less than 7 g/dL occurred in one (0·1%) of 1208 patients treated without primaquine, none of 893 patients treated with a low daily dose of primaquine (<0·375 mg/kg per day), five (0·3%) of 1464 patients treated with an intermediate daily dose (0·375 mg/kg per day to <0·75 mg/kg per day), and six (0·5%) of 1269 patients treated with a high daily dose (≥0·75 mg/kg per day). The covariate-adjusted mean estimated haemoglobin changes at days 2-3 were -0·6 g/dL (95% CI -0·7 to -0·5), -0·7 g/dL (-0·8 to -0·5), -0·6 g/dL (-0·7 to -0·4), and -0·5 g/dL (-0·7 to -0·4), respectively. In 51 patients with G6PD activity between 30% and less than 70%, the adjusted mean haemoglobin concentration on days 2-3 decreased as G6PD activity decreased; two patients in this group who were treated with a high daily dose of primaquine had a reduction of more than 25% to a concentration of less than 7 g/dL. 17 of 18 included studies had a low or unclear risk of bias.

    INTERPRETATION: Treatment of patients with G6PD activity of 30% or higher with 0·25-0·5 mg/kg per day primaquine regimens and patients with G6PD activity of 70% or higher with 0·25-1 mg/kg per day regimens were associated with similar risks of haemolysis to those in patients treated without primaquine, supporting the safe use of primaquine radical cure at these doses.

    FUNDING: Australian National Health and Medical Research Council, Bill & Melinda Gates Foundation, and Medicines for Malaria Venture.

    Matched MeSH terms: Hemoglobins
  19. Yamsri S, Kawon W, Duereh A, Fucharoen G, Fucharoen S
    J Pediatr Hematol Oncol, 2021 04 01;43(3):e341-e345.
    PMID: 32815885 DOI: 10.1097/MPH.0000000000001920
    OBJECTIVES: Southeast Asian ovalocytosis (SAO) is an inherited red blood cell (RBC) membrane disorder, whereas hemoglobinopathies are inherited globin gene disorders. In an area where both diseases are prevalent, the interaction between them resulting in variable hematologic parameters can be encountered. However, little is known about the genetic interaction of SAO and thalassemia. We investigated the prevalence of SAO and hemoglobinopathy genotypes among newborns in southern Thailand.

    PATIENTS AND METHODS: This study was carried out on 297 newborns recruited consecutively at Naradhiwas Rajanagarindra Hospital in the south of Thailand. The SAO was identified on blood smear examination and polymerase chain reaction analysis. Thalassemia genotypes were defined. Hematologic parameters and hemoglobin (Hb) profiles were recorded and analyzed.

    RESULTS: Among 297 newborns, 15 (5.1%) carried SAO, whereas 70 (23.6%) had thalassemia with 15 different thalassemia genotypes. Abnormal Hb including Hb C, Hb Q-Thailand, and Hb D-Punjab were observed in 5 newborns. It was found in the nonthalassemic newborns that RBC count, Hb, and hematocrit of the nonthalassemic newborns with SAO were significantly lower than those without SAO. The same finding was also observed in the thalassemic newborns; RBC count, Hb, and hematocrit of the thalassemic newborns with SAO were significantly lower than those without SAO. However, the mean corpuscular volume, mean corpuscular Hb, and RBC distribution width of the SAO-newborns were significantly higher.

    CONCLUSIONS: Both SAO and hemoglobinopathy genotypes are common in southern Thailand. One should take this into consideration when evaluating neonatal anemia and other hematologic abnormalities. Identification of both genetic defects and long-term monitoring on the clinical outcome of this genetic interaction should be essential to understand the pathogenesis of these common genetic disorders in the region.

    Matched MeSH terms: Hemoglobins, Abnormal/analysis; Hemoglobins, Abnormal/genetics
  20. Ong HC
    Acta Haematol., 1974;52(4):220-2.
    PMID: 4217527 DOI: 10.1159/000208244
    Haemoglobin E complicates 22.2°/o of pregnancy in Malaysian aborigines, the prevalence of variants associated with pregnancy being, 15.8% with Hb E trait abnormality, 3.9% with Hb E homozygous disease, and 2.5% with Hb E thalassaemia disease. Minor haematological abnormalities occur with the trait and homozygous conditions, though a more unfavourable response is expected with Hb E thalassaemia. Haemolysis is not a prominent feature and it is suggested that factors other than the haemoglobinopathic state
    probably accounts for any unfavourable response in pregnancy.
    Key Words: Haemoglobin E; Haemoglobinopathies; Haemolytic anaemias; Hb E thalassaemia; Malaysia; Pregnancy
    Study site: Hospital Orang Asli, Gombak, Selangor, Malaysia
    Matched MeSH terms: Hemoglobins; Hemoglobins, Abnormal*
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