Haemoglobin Bart's hydrops fetalis syndrome is totally lethal. Globin chain electrophoresis on mylar backed cellulose acetate strips, by a method modified from Ueda and Schneider has been established to demonstrate total absence of alpha chains in this syndrome. This simple test can identify fetuses, stillbirths and newborns with homozygous αo-thalassaemia. In this region where DNA studies are limited, and prenatal diagnosis is unavailable, this test which describes the phenotypic expression of Hb Bart's syndrome will improve genetic counselling of women at risk of homozygous αo-thalassaemia.
Haematologic abnormalities are found in patients who develop acute ischaemic syndromes including acute myocardial infarction (AMI). A coronary care unit population of 660 consecutive patients were studied for their haematologic parameters on admission. There were 263 patients who had AMI, 207 who had non-infarct acute ischaemic syndromes (CAD), and the other 190 non-coronary patients served as controls. Overall patients with AMI had signijicantly higher haemoglobin (145 ± 2.2 vs 12.9 ± 3.0 g/dl, p<0.0001), haematocrit (45.4 ± 5.8% vs 41.2 ± 8.8%. P < 0.001). red cell count (5.0 ± 0.7 vs 4.5 ± 10^12 p<0.001), but there were no significant differences in their leucocyte count, platelet count or mean corpuscular volume. Patients with CAD had higher haemoglobin and red cell counts (p<0.002, p<0.02 respectively). Although infarct women tended to have lower counts (p<0.001), they still had significantly higher haemoglobin (p<0.01), haematocrit (p<0.02) and red cell count(p<0.002) when compared with controls. Interestingly, there were no ethnic differences at all with regard to haematologic values for infarct patients; haemoglobin, haematocrit, and red count were each significantly higher than controls. Our results suggest that a relatively high baseline Hb, haematocrit or RBC count may be associated with increased likelihood of acute ischaemic syndromes including AMI among Malaysians. Increased viscosity and abnormal haemorheological characteristics may contribute to the development and subsequent outcome of unstable coronary syndromes. Keywords: acute myocardial infarction, coronary artery disease, haemoglobin, haematocrit, haemorheology.
Introduction: It is mandatory that every blood donor must have their haemoglobin (Hb) values measured before blood donation. High Hb may indicate an underlying hidden pathological condition. The aim of this study is to investigate the occurrence of the JAK2 V617F gene mutation in blood donors with erythrocytosis. Methods: A cross-sectional study was conducted over a nine-month period involving blood donors with high pre-donation Hb. A total of 45 blood donors with total white cell (TWC) > 12.0x 109/l, platelet > 450x109/ l and Hb > 18g/dL were subjected to JAK2 V617F gene mutation analysis. Samples were collected and analysed for haematological tests and detection of JAK2 V617F mutation. Results: From a total of 2238 blood donors, 175 blood donors had high haemoglobin value. Samples from forty-five of these donors were then analysed for JAK2 V617F using allele-specific polymerase chain reaction (PCR). The prevalence of blood donors with erythrocytosis was 7.8%. All samples were negative for the JAK2 V617F mutation. Conclusions: Erythrocytosis can be relative or absolute and the different causes can be distinguished on the basis of clinical signs and symptoms. An absence of the JAK2 V617F mutation cannot by itself excludes the diagnosis of polycyhaemia vera (PV) since erythrocytosis is the single clinical feature that sets PV apart from other types of myeloproliferative neoplasm (MPN). Further study is required for the detection of other gene mutations that activates the JAK-STAT signalling pathway that could be identified in JAK2 V617F-negative MPN patients.
Introduction: Reduction or complete absence of α-globin chain production may result α-thalassemia. Alpha thalassemia carrier may have normal haemoglobin level and thus will be eligible as blood donor. Few complications may happen in which the carrier who donated the blood might be at risk of hypoxia and their blood components might not suitable for transfusion. Thus, it is important to screen for α-thalassemia to prevent any complications happen
after donation. The objective of this study is to investigate the interaction of red blood cell indices and α-globin genotypes among eligible blood donors in a private university, Universiti Tunku Abdul Rahman (UTAR), Malaysia. Methods: A total of 270 eligible blood donors were recruited for this study. Red cell indices were analysed using Horiba hematology analyser and α-globin genotyping was performed for seven alpha deletions, six alpha point mutations
and two alpha triplications. Results: Our study showed high prevalence of α-thalassemia carriers among the eligible blood donors (7.7%, 21/270), with all of them showed normal Hb level (>12 gm/dl). Five genotypes were detected consisting of 249 αα/αα (92.2%), 9 -α3.7/αα (3.3%), 9 --SEA/αα (3.3%), 2 -α4.2/αα (0.7%) and 1 ααCS/αα (0.4%). All α-globin genotypes showed normal Hb level with no significant difference between genotypes (p=0.167). Different
α-globin genotypes showed significant difference in RBC, MCV, MCH, MCHC, RDW and Hct/Hb ratio at the p
This research was conducted to estimate the effects of Persicaria odorata leaf meal (POLM) on haematological indices, serum biochemical attributes, and internal organs parameters, including histomorphological features of the liver, in broiler chickens. A total of 120 one-day-old male broiler chicks (Cobb-500) were randomly allocated into four experimental groups. The dietary treatments were basal diet (BD), which served as the control (C), along with BD + 2 g/kg POLM (Po2), BD + 4 g/kg POLM (Po4), BD + 8 g/kg POLM (Po8), which were the supplemented groups. The body weight gain (BWG) showed a linear increase and feed conversion ratio (FCR) showed a linear decrease with increasing POLM dosage at day 42 (p ˂ 0.05) and for the overall growth performance period (p ˂ 0.01). On day 21 and day 42, the values of red blood cells (RBCs), white blood cells (WBCs), haemoglobin (Hb), and packed cell volume (PCV) showed linear increases (p ˂0.05) as the dosage of POLM increased in the diet. On day 21, dietary supplementation of POLM linearly decreased (p ˂ 0.05) the serum activity of alkaline phosphatase (ALP), aspartate aminotransaminase (AST), alanine aminotransaminase (ALT), and serum levels of urea and creatinine. On the other hand, serum levels of total protein (TP), albumin, and globulin showed a linear increase (p ˂ 0.05) as the POLM dosage increased. On day 42, the serum activity of AST and ALT and serum levels of glucose, cholesterol, triglycerides, urea, and creatinine showed linear decreases (p ˂ 0.05) with increased levels of POLM in the diet. However, POLM supplementation linearly increased (p ˂ 0.05) the serum levels of TP and globulin. Dietary inclusion of POLM did not influence the organ parameters and showed no adverse effects on the liver histomorphology. In conclusion, supplementation of POLM increased the growth performance, improving haematological indices and serum biochemistry profiles of broiler chickens without any deleterious effects on the liver histomorphology. The results of the present study provide evidence that POLM can be safely used at a dose rate of 8 g/kg of feed as an alternative to conventional antimicrobial growth promoters (AGPs).
This study was carried out to determine the nutritional status of 16 elite netball players of the Commonwealth Games 1998, during centralised training at Bukit Jalil. The mean age of the subjects was 21.1 ± 3.1 year. The physical characteristics were assessed through antropometric measurements. The dietary intake was estimated as a mean of 3-days weighed food record while the physical activity pattern was determined by time and motion study which was carried out simultaneously. A biochemical analysis test was carried out to evaluate the haemoglobin status of the athletes. The results indicated that the mean body weight and height were 62.5 ± 7.9 kg and 1.69 ± 0.06 meter, respectively. The percentage average body fat of the subjects was 23.7 ± 2.8. Mean energy intake was recorded as 2726 ± 507 kcal/day while energy expenditure was 3225 ± 409 kcal/d with a negative energy balance of 499 kcal. The contribution of calories from carbohydrate, protein and fat were 59%, 16% and 26%, respectively. Intake of most micronutrients was sufficient and met the Malaysian RDA. The percentage average of the daily physical activity pattern revealed that 74% was spent for light activities, 12% for moderate while 14% for heavy activities. 94% of the athletes took supplements which vitamin C was the most popular (64%).
This is the first report of QQQ-mass spectrometric identification and quantification of the Hb subunits, alpha, beta, delta and gamma globin peptides, derived from enzymatic-digestion of proteins in the early unknown peaks of the Bio-Rad cation-exchange chromatography of haemoglobin. The objectives were to assess the relationship of the quantity of the free alpha, beta, delta and gamma globin chains with the phenotypic diversity of beta-thalassaemias (β-thal). The results demonstrate that the pools of free globin chains in red blood cells were correlating with the severity of the disease in patients with different phenotypes of β-thal. The mechanism and the regulation of synthesis of free globin chains pool in a normal individual and in patients with different β-thal phenotypes could arise from several mechanisms which will require further investigation. The role of the free globin pool in patients with β-thal for development of novel therapeutic approaches based on these potential targets requires further investigation. Pertinent biomarkers improves the diagnosis of the β-thal, especially in low-income countries where they are most common and allows more effective therapeutic intervention leading to more successful therapeutic outcome.
Introduction: Operative laparoscopy is the gold standard approach for treatment of tubal pregnancy. Although benefits of this approach are well established, data on its uptake trend in Malaysia is largely unknown. Objective: This study aims to determine the operative laparoscopy uptake in management of tubal pregnancy at a busy tertiary hospital and whether the benefits associated with laparoscopic surgery was achieved. Materials and Methods: This prospective observational study was conducted on all women admitted for tubal pregnancy at Hospital Sultanah Aminah, Johor Bahru, a public tertiary hospital over a period of 12 months. The on-call team was responsible for the surgical approach. Patient’s clinical presentation, operative laparoscopic uptake, factors affecting the choice of approach and duration of hospitalization were analyzed. Results: The tubal pregnancy rate was 7.6 per 1000 deliveries. Twenty- seven of the 138 cases (20%) had hypovolemic shock, requiring urgent laparotomy and were excluded from study. The operative laparoscopy rate for stable tubal pregnancy was only 42.3% (47 of 111 cases). Women managed laparoscopically were associated with a significantly higher pre-operative hemoglobin level, mostly nullipara and had surgery performed during office hours. They waited longer for their surgery but were discharged earlier compared to the laparotomy group. There was no difference in the duration of hospitalization. Conclusions: Less than half of all hemodynamically stable tubal pregnancies in our hospital had operative laparoscopy. The current laparoscopy uptake rate can be further improved.
Introduction: Thalassaemia is an inherited blood disorder and is a significant public health alarm in Malaysia with many not knowing they are carriers of this haemoglobin disorders. Materials and methods: This study conducted a one off collection of blood samples from 72 Malays students of International Islamic University Malaysia (IIUM) in Kuantan. Blood samples were subjected to conventional haemoglobin analyses that include full blood count and picture, HPLC, Haemoglobin electrophoresis and H-inclusion test. All samples were also genotyped for alpha thalassaemia–1 of Southeast Asia (a-Thal1SEA). Result: There were 17(23.6%) students who were diagnosed as thalassaemia carriers. Out of this, four (5.5 %) and six (8.3 %) students were presumptive β-thalassaemia trait and Haemoglobin-E trait as determined by the HPLC assay respectively. Nine (12.5%) students were genotyped a-Thal1SEA among whom two were also β-thalassaemia carriers. All thalassaemia cases had MCH of < 27pg. Nonetheless, two out of six Haemoglobin-E trait and three out of nine a-Thal1SEA carrier had MCV value of >80fL. Two out of four (50%) presumptive β -thalassaemia trait and one out of six (17%) students of presumptive Haemoglobin-E trait had family history of thalassaemia respectively. Conclusion: The high occurrence of the three common types of thalassaemia carrier (β, Hb-E and a-Thal1SEA thalassaemia) in our small group of subjects could be due to better participation of students who had family history of thalassaemia. The study reaffirmed the importance of molecular study for detection of alpha-thalassaemia and the use of MCH value of
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme deficiency worldwide including Malaysia. Screening of cord blood for partial G6PD deficiency is important as they are also prone to develop acute haemolysis. In this study, we determined the prevalence of partial G6PD deficient in paediatric population aged 1 month-12 years and normal term female neonates using OSMMR-D kit with haemoglobin (Hb) normalization and compare it with florescence spot test (FST). A total of 236 children, aged between between 1 month-12 years and 614 normal term female neonates were recruited for this study. Determination of normal means for G6PD activity and; cut-off points for partial and severe deficiency were determined according to WHO Working Group (1989). Determination of prevalence for partial deficiency for both groups (female patient) was done using this enzyme assay kit and findings were compared with FST. In this study, 15.7% (18/115) female children were classified as partial G6PD deficient by quantitative enzyme method (G6PD activity: 4.23-5.26U/gHb). However, FST only detected 0.9% (1/115) with minimal G6PD activity. The prevalence of partial G6PD deficiency in female neonate group was 3.42% (21/614) by enzyme assay versus 0.49% (3/614) by FST. This study concluded that our routine screening method using FST was unable to diagnose female heterozygotes. We recommend using this quantitative enzyme assay method by OSMMR-D kit since it was more sensitive in detecting G6PD deficiency in female neonates compared to FST.
Introduction: Anemia in pregnancy is still a public health problem in developing countries including Malaysia. Early screening of pregnant mothers who have risk factors of anemia could help identifying these potential anemic mothers and hence targeted for intervention.
Methodology: A cross sectional study IN 2008 was conducted among pregnant women who attended government health clinics in Johor Bahru district to assess the prevalence of anemia in pregnancy and factors associated with it using a structured questionnaire.
Result: Prevalence of anemia in pregnancy (Hb<11.0 g/dl) was 36.6% and majority in mild category (Hb 9-<11 g/dl). The associated factors of anemia in pregnancy were birth spacing, dietary intake with high iron content and iron pill consumption. Multivariate analyses revealed that iron pill intake was the most important factor influencing anemia in pregnancy.
Discussion and conclusion: Even though Johor Bahru is a big town with good health facilities, the prevalence of anemia in pregnancy was quite high. Results were compared with previous studies. Emphasis on compliance to iron pills is very important in prevention and treating anemia in pregnancy
Key Words: anemia in pregnancy, birth spacing and iron pill intake
Study site: Four urban and two rural klinik kesihatan, Johor, Malaysia
Objective: To assess the prevalence of iron deficiency, anaemia and iron deficiency anaemia (IDA) in women aged 20-40 years and its association with iron intake. Methods: A total of 388 women were recruited from universities and work sites in Kuala Lumpur and its suburbs. The subjects comprised 135 Malays, 130 Chinese and 123 Indians. Dietary intake was estimated using a single 24-hour food recall and a semi-quantitative food frequency questionnaire. Haemoglobin (Hb), haematocrit (hct), mean corpuscular volume (MCV) and serum ferritin were determined. Results: The prevalence of anaemia (Hb
Objectives: This study aimed to evaluate the UBI MAGIWELTM ζ-GLOBIN ELISA Kit for the presumptive diagnosis of αo-thalassaemia. The ELISA results obtained were confirmed by molecular characterisation of αo-thalassaemia using a Duplex-PCR. Methods: Routine peripheral blood counts and red cell indices were determined in 94 blood samples sent for Hb analysis. Hb subtypes were quantified by high performance liquid chromatography (HPLC) and Hb electrophoresis conducted on agarose gel at pH 8.5. Zeta-globin chain levels were determined using the UBI MAGIWELTM ζ-GLOBIN ELISA Kit. Molecular analysis was performed using a duplex-PCR which simultaneously amplifies
a normal 136 bp sequence between the ψα−α2-globin genes and a 730 bp Southeast Asian deletion-specific sequence (–SEA) between the ψα2−θ1-globin genes. Results: Using the ELISA assay kit, 20 blood samples were presumptively identified as α-thalassaemia carriers from elevated ζ-globin chains (OD>0.3) while the remaining 74 blood samples showed OD
We report here a case of severe iron deficiency anaemia resulting from prolonged exclusive milk diet without any iron supplementation. The patient was a 1112 year old boy, the only child of a factory worker, who was breasffed until 4 months of age followed by exclusive feeding by non-fortifiedformula. He presented with severe iron deficiency anaemia with haemoglobin of4 .0g% and required blood trans-fusion. Iron and.vitamin supplements were given and cereals were slowly introduced. He responded very well to the treatment with satis-factory weight gain and improvement of anaemia. (Copied from article).
Thalassaemia screening programme has been conducted in Malaysia since 2004. The aim of the programme was to reduce the burden of the disease by identifying thalassaemia carriers. However, the response towards the screening activities was unsatisfactory as there was lack of public awareness against the importance of thalassaemia screening. An alternative approach is to screen blood donors. The purpose of this study was to observe the prevalence of thalassaemia carriers among healthy blood donors. Seven hundred and thirty eight healthy blood donors were screened in Hospital Tengku Ampuan Rahimah, Klang from July to September 2010 using cation-exchange high performance liquid chromatography (HPLC). Cases with haemoglobin variants were further analyzed by gel electrophoresis at alkaline pH. Result shows that the blood donors consisted of 413 Malays (56%), 162 Indians (22%), 148 Chinese (20%) and 15 others (2%). There were 19 (2.6%) individuals with haemoglobin E trait, six (0.8%) with co-inheritance of haemoglobin E and αα- thalassaemia and five (0.7%) with β-thalassaemia trait. Haemoglobin Constant Spring and haemoglobin A2 prime were observed in two (0.3%); and Haemoglobin Lepore and alpha chain variant in one (0.2%). αα-thalassaemia and normal haemoglobin A2 β-thalassaemia could not be excluded in 190 cases (26%), as they required deoxyribonucleic acid (DNA) studies for identification. Thalassaemia screening in blood donors is more feasible and effective. Therefore, a wider scale population screening including blood donors could benefit the existing thalassaemia screening programme in Malaysia.
A 65-year-old lady with cholelithiasis underwent an elective laparoscopic cholecystectomy (LC) and was discharged on post-operative day 2. She was re-admitted after five days with right hypochondriac pain and fever. Investigations revealed a drop in hemoglobin. Computed tomography showed a large intrahepatic subcapsular collection without intra-abdominal collection. She was treated with intravenous antibiotics. Percutaneous aspiration of intrahepatic subcapsular collection under ultrasound guidance revealed old blood. Hence diagnosis of intrahepatic subcapsular hematoma (ISH) was made. She improved and was discharged after a two weeks' hospital stay. Follow-up ultrasonography examination two months later revealed complete resolution of the hematoma. We report this case due to its rarity and review the previously documented cases of this complication.
Haemoglobin E (Hb E) is a variant of structurally abnormal haemoglobin that can be found very commonly in the Asian countries particularly the Southeast Asian [1]. [H1] Alpha thalassaemia is a red cell disorder which is caused by deletion or mutation of one or more of the four alpha globin genes leading to absence or decrease in production of alpha globin peptides [2]. This disorder is far more common in South East Asian regions and in Malaysia itself, and the gene frequency is about 4.1% [2]. The interactions of Hb E and alpha thalassaemia are evident in Kelantan which is bordered by southern Thailand. Using capillary electrophoresis (CE), a reduction of Hb E level is noticed as compared to Hb E heterozygotes. DNA analysis should be done to determine the presence of concurrent alpha thalassaemia variant. This study was done to evaluate haematological parameters using automated blood counters, morphology of red cells, Hb separation and quantitation of Hb fractions using CE and molecular analysis for alpha thalassemia. The study also aimed to discover cut off point of Hb E level in heterozygous Hb E patients with concurrent deletional alpha thalassaemia by CE.
The end stage renal disease (ESRD) patients requires hemodialysis to survive. Efficacy of the treatment is determined by evaluation of minimal dialysis dose (Kt/V) which is 1.2. A cross sectional study was conducted among patients that undergo hemodialysis in a dialysis centre in Kuala Lumpur, Malaysia. The objectives of the study were to determine the association between dialysis dose and demographic factors and assessed the association between biochemical blood parameter and the demographic factors. The biochemical blood parameters were serum albumin, creatinine, cholesterol and hemoglobin. Result showed that all Indians and 54% of Chinese patients achieved the required dialysis dose. However only 29% of Malay patients attained the effective dialysis dose. More women patients accomplished the dialysis dose of at least 1.2 compared to men patients with odd ratio of 11.24. All the biochemical blood parameters were independent of the demographic factors. However, the cholesterol level was associated significantly with gender (p<0.05). In conclusion, the study found the biochemical blood parameter and dialysis dose were not influenced by the demographic factors.
HbE/Beta thalassemia (HbE/β-thalassemia) is one of the common genetic disorders in South East Asia. It is heterogeneous in its clinical presentation and molecular defects. There are genetic modifiers which have been reported to influence the disease severity of this disorder. The aim of this study was to determine the genetic polymorphisms which were responsible for the disease clinical diversity. A case-control study was conducted among Malay transfusion-dependent HbE/β-thalassemia patients. Patients who were confirmed HbE/β-thalassemia were recruited and genotyping study was performed on these subjects. Ninety-eight patients were selected and divided into moderate and severe groups based on clinical parameters using Sripichai scoring system (based on hemoglobin level, spleen size, growth development, the age of first transfusion and age of disease presentation). Forty-three (44.9%) and 55 (56.1%) patients were found to have moderate and severe clinical presentation, respectively. Genotyping analysis was performed using Affymetrix 6.0 microarray platform. The SNPs were filtered using PLINK and Manhattan plot by R software. From the GWAS results, 20 most significant SNPs were selected based on disease severity when compared between moderate and severe groups. The significant SNPs found in this study were mostly related to thalassemia complications such as rs7372408, associated with KCNMB2-AS1 and SNPs associated with disease severity. These findings could be used as genetic predictors in managing patients with HbE/β-thalassemia and served as platform for future study.