The beta-thalassaemia mutations in 20 Malaysian children with beta-thalassaemia major were characterised by using a multi-modal approach, consisting of a slot-blot hybridisation with selected allele-specific oligonucleotides (ASO), followed by reverse dot-blot assay (RDB), amplification refractory mutation system (ARMS) and genomic sequencing. This strategy yielded a 94.4% mutation detection rate. The 6 most common mutations were codons 41/42 (-TTCT), IVS II nt 654(C --> T), IVS I nt 5(G --> C), IVS I nt 1(G -->T), codon 35 (-C) and codon 19 (A --> G), which accounted for 83.3% of all mutations detected. A strategy of initial screening with the above 6 selected ASOs for slot-blot hybridisation followed by RDB assay for the less common Asian mutations would give a mutation identification of 91.7%. Another feasible approach would be to analyse alleles from a particular racial group, by a judicious selection of 4 ASOs common to that particular subpopulation and then supplement this with RDB assay. This could yield a 100% coverage for the Chinese subpopulation in Malaysia. With these strategies, a practical approach has been identified to overcome the pitfalls posed by the molecular heterogeneity of beta-thalassaemia to enable prenatal diagnosis and carrier screening to be carried out. Regional collaborative studies are to be encouraged as an indispensable tool in providing better health care services to our patients.
A cross-sectional study on the expression of 6 lymphocyte markers was carried out on 481 patients with human immunodeficiency virus (HIV) and 79 normals after stratification based on absolute CD4 counts. The data were stratified according to the following groups: (I) 1201 to 1600, (II) 801 to 1200, (III) 401 to 800 and (IV) 0 to 400 (x 10(6) CD4 cells per mm3). The mean percentages of the subsets before stratification showed that HIV patients had increased percentages of CD3+ (75.7 against 66.9), CD3+CD8+ (52.2 against 32.3) and CD3+HLA-DR+ (36.1 against 14.4) cells and lower percentages of CD19 (10.3 against 13.3) and natural killer cells (13.7 against 20.4) when compared to controls in the same group. A definite trend, however, was only seen in CD3+CD8+ (47.4, 50.0, 54.0, 57.5 for groups I, II, III and IV respectively) and CD3+HLA-DR+ (29.1, 32.9, 38.4, 43.9 for groups I, II, III and IV respectively).
This paper presents data on two surveys done in 1989 and 1992 that indicated the attitudes of Singaporeans to sexuality. Both surveys revealed that Singaporeans have sexually conservative attitudes and those who have a lower education, are single, above the age of 40, are Malays/Muslims hold more conservative views.
General life tables for the 1990 Singapore resident population are given in this paper. Analyses were carried out separately for males and females for all ethnic groups as well as for Chinese, Malays and Indians. Average fractions of the last age interval lived were used to give a more precise derivation of the life table death rates from their corresponding age-specific death rates. The results show that in 1990, Singapore males had achieved a life expectancy at birth of 73.7 years while the females had attained a level of 78.6 years. These levels were comparable to those of many developed countries. Other interesting features include higher differentials in the life expectancies between sexes among the Chinese and the Indians taking over the Malays for second placing in terms of life expectancy at birth for both sexes. As a result, the Indians registered a 12% gain in life expectancy at birth for males for the decade 1980 to 1990, compared to only 7.1% for Chinese and 4.4% for Malays as well as nearly 10% for females compared to only 6.2% for Chinese and 6.3% for Malays. A subsequent paper will highlight the results of multiple-decrement life table analyses which will summarise the mortality of the 1990 Singapore resident population for various component causes of death rather than for all causes combined.
Drug-induced agranulocytosis is a potentially fatal complication despite advances in supportive care. A patient with carbimazole-induced agranulocytosis associated with marked depletion of granulocytic precursors in the marrow was treated with granulocyte-macrophage colony stimulating factor (GM-CSF) at a dose of 5 micrograms/kg subcutaneously daily for one week. The absolute neutrophil count rose above 1 x 10(9)/L after one week of GM-CSF therapy. The GM-CSF probably expedited the recovery of the neutrophil count. Further studies are warranted to delineate the role of GM-CSF in the treatment of drug-induced agranulocytosis.
A typical case of chronic pericardial effusion resulting in cardiac tamponade is presented. A pericardiocentesis was done for diagnosis and drainage, followed by a pleuro-pericardial window as definitive therapy. The minimal cumulative dose expected to produce pericardial disease is about 4000 rads, and the disease usually manifests within 12 months of such radiation exposure, as in this patient. It is concluded that for symptomatic pericardial effusions, available evidence justifies a subtotal pericardiectomy, a window procedure being reserved to tide over ill patients as in this patient. No strong evidence exists for the efficacy of steroid therapy; such therapy is reserved for asymptomatic mild effusions, which may also resolve spontaneously.
Thyroid function and pubertal development of aborigines (Orang Asli) and Malays at different socioeconomic strata were assessed among 1136 subjects aged 7 years and above. Anthropometric measurements, goitre and pubertal staging were done. Serum thyroxine (T4), triiodothyronine (T3) and growth hormone were measured using radioimmunoassays (RIA) and serum thyroid stimulating hormone (TSH) by immunoradiometric assays (IRMA). It was found that serum T3 in children was significantly higher in Malays from rural areas, girls and children aged less than 13 years. However, in adults, T3 was significantly associated with anthropometric indices. On the contrary, serum T4 levels were higher among children from urban areas. In adults, serum T4 levels were significantly related to nutritional status and they increased according to the levels of social development, being lowest in remote areas and highest in urban areas. However, serum TSH levels were significantly higher in Orang Asli at all ages and among malnourished children. By using multiple regression, apart from age, gender and ethnicity, nutritional status was a significant predictor for T3 levels in children and adults. Presence of goitre was an important factor which determined the T4 levels in children and adults after controlling for other factors. It was also a predictor for TSH levels in children but not in adults. Fasting serum growth hormone (GH) levels were significantly higher among less privileged groups and decreased according to social development. Serum growth hormone was negatively correlated with anthropometric indices and had a significant association with malnutrition.(ABSTRACT TRUNCATED AT 250 WORDS)
We describe two Malay male term neonates with congenital limb reduction defects. The first neonate had hypodactyly of limbs associated with micrognathia, microstomia, glossopalatine ankylosis and congenital mitral stenosis. He developed gram-negative septicaemia and died on day 14 of life. The second neonate had tetraperomelia without any other associated congenital abnormality. He developed staphylococcal skin infection which was treated conservatively. Very few cases of congenital limb reduction defects have been reported in the Asian population and we are not aware of any other reports describing Malay infants with the congenital abnormalities described in this report.
The management of haemorrhagic episodes in patients with factor VIII inhibitor is difficult and the outcome rather unpredictable. The use of an investigational drug, that is, activated recombinant factor VII (rFVIIa) in a young non-haemophiliac patient with spontaneous occurrence of factor VIII inhibitor who presented with life-threatening retroperitoneal haemorrhage is reported. There was prompt achievement of haemostasis with rFVIIa after the patient had failed conventional therapy with factor IX and Autoplex. Two further episodes of retroperitoneal bleeding again responded promptly to rFVIIa therapy.
A systematic collection of fetal morphometric measurements was started seven years ago in 1987 with the prospective entry of data into personal computer-based stations in the Department of Obstetrics and Gynaecology, Singapore General Hospital. A cross-sectional study was done, comparing 2392 Chinese fetuses with 2117 Malay fetuses and 459 Indian fetuses from 18 to 40 weeks. The mean values of the head circumference (HC), abdominal circumference (AC) and femur length (FL) of the 3 ethnic groups were analysed. There were no statistical significant differences in the head circumference and abdominal circumference of Chinese, Malay and Indian fetuses in Singapore. The Chinese and Malay fetal femur length appeared similar but were apparently shorter than the Indian femur length. Nomograms of head circumference, abdominal circumference and femur length were constructed for application to fetuses of all 3 ethnic groups.
A cross-sectional survey of 568 married women aged 21-65 years in the Pasir Panjang Constituency was conducted to determine the prevalence of cervical cancer screening in the community and the factors affecting acceptance of this screening test. Results show that 309 (54.4%) of the respondents had ever had a Pap smear. The women who reported having had a smear were more likely to be below 45 years of age (prevalence ratio 1.47, 95% confidence interval 1.15 to 1.88), Chinese and of a higher socioeconomic status (1.68, 1.33 to 2.12). They were also more likely to have married at a later age (1.68, 1.21 to 2.31) than women who had not had a smear. Age and socioeconomic status remained independent predictors on multivariate analysis. The findings suggest that an important task of public health practitioners is to increase the uptake of screening among older and socially disadvantaged women who are known to be at the highest risk of cervical cancer. In addition, strategies to encourage younger women to continue regular attendance at screening clinics beyond their childbearing years will be important for the future.
The Programme on Prevention and Management of Obesity in Preschool Children, aged three to six years, was implemented in 17 Primary Health Clinics in November 1991. The study sample comprised 1128 preschool children who qualified to enter the obesity register, using the defined criteria for obesity of 2 standard deviations above the normal weight for height and age. This group was divided into three categories, namely, mildly overweight (120% to below 140%), moderately overweight (140% to below 160%), and severely overweight (above 160%). The severely overweight category was referred to dietitians for follow-up management, while the other two categories were managed by the clinic staff through pre-planned nurse-conducted counselling sessions. In this paper, we analysed the first 1128 preschool children aged three to six years on the Programme with respect to their demographic characteristics; medical conditions; family history of obesity, diabetes, hypertension and ischaemic heart disease; number of siblings and parents' educational and occupational status. The Malay children showed significantly more severe grades of obesity compared to the Chinese and Indian children. A family history of obesity and hypertension among the three groups were significant (P < 0.001). After one year of follow-up with the intervention programme, the following were found: 40.4% (456) of the children improved in their obesity status and 20.2% (228) reached normal status. The severe, moderate and mild categories reduced from 6.3% to 5.9%, 29.3% to 23.2% and 64.4% to 50.7% respectively and was found to be statistically significant (P < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)
The clinical severity of the mutations causing beta-thalassaemia in West Malaysia is presented. Thalassaemia clinical scores (Thal CS), a scoring system, has been formulated to predict clinical severity. It is the type of beta-thalassaemia mutation present that decides on the clinical phenotype. The most severe beta-thalassaemia mutation is assigned a score of 4. A score of 8 indicates a severe thalassaemia phenotype. Alpha-thalassaemia, increased synthesis of Hb F, and glucose-6-phosphate deficiency may ameliorate the clinical condition at phenotype level, and the co-inheritance of hereditary ovalocytosis aggravates it.
We conducted a population-based study involving a stratified cluster disproportionate random sample of 2868 adults aged 20 to 74 years selected from five housing estates (Yishun, Toa Payoh, Jurong East, Geylang/Eunos and Bukit Merah). Chronic rhinitis was defined as the usual presence of symptoms of blocked or running nose, apart from colds or the flu, lasting for more than a year. Allergic rhinitis was considered to be present if these symptoms were associated with conjunctivitis or recognisable provocation by commonly known allergens, namely house dust, dogs, cats, birds, pollen, or medicines. The estimated general population prevalence of chronic rhinitis was 10.8% (95% confidence interval (CI) 8.8-12.7). Higher prevalences were noted in males, in younger adults, in Indians and Chinese, in those with higher socio-economic status, and in Toa Payoh, Jurong East and Geylang/Eunos. The prevalence of allergic rhinitis was 5.5% (95% CI 4.5-6.5). The most common 'allergenic' factor in allergic rhinitis was house dust (73%); provocation by birds, cats or dogs (5%), grass or tree pollens (5%), and medicine (5%) was less common but likely to have been under-recognised. Provocation by change in temperature (54%) and early in the morning (64%) was equally frequent in both 'allergic' and 'non-allergic' rhinitis; cigarette smoke, petrol and diesel fumes, food and work environment were less common (4-12%). Allergic rhinitis was highly significantly associated with asthma (13.9% in allergic rhinitis, 4.4% in non-allergic rhinitis, and 2.2% in non-rhinitic subjects).
Four hundred and one cases of ameloblastoma of the jaw diagnosed in the Division of Stomatology, Institute for Medical Research, Kuala Lumpur, between 1967 and 1991 have been analysed. There were 214 males and 187 females. Of these, 50.1% were Malays, 34.7% Chinese, 8.2% Indians and 7.0% other races. Seventy-two percent of patients were in the second, third and fourth decades of life (mean age: 30.8 years). Ninety-three percent of tumours occurred in the mandible and 6.5% in the maxilla. There were 337 cases of conventional ameloblastoma, 49 cases of the unicystic variant and four cases of peripheral ameloblastoma. The predominant histologic patterns were plexiform (34.2%), follicular (16.5%) and mixture of both (17.7%). Majority of the cases were conservatively treated by enucleation. Fifty-nine cases presented with recurrences. Present findings were generally compatible with regional serial studies reported from Thailand, Singapore and Japan.
Forced vital capacity (FVC), forced expiratory volume in one second (FEV1), peak expiratory flow rate (PEFR), single-breath diffusion capacity measurements (effective alveolar volume (VA), carbon monoxide transfer factor (DLCO) and transfer coefficient (KCO)) were determined in 452 healthy Singaporean adults (277 males and 175 females) aged 20-70 years. The ratio of Chinese, Malay and Indian subjects was 5:2:3 in both sexes. Age, height and weight in the males were all significantly correlated with FEV1, FVC, DLCO, VA and PEFR. However, for females, only age and height were significantly correlated with the studied lung function parameters. Significant ethnic differences were observed for most of the pulmonary functions (except KCO and PEFR) among the Chinese, Malays and Indians for both males and females. The predicted FEV1 and FVC values (specific age and height) for both sexes were highest among the Chinese followed by the Malays than Indians, in that order. Regression equations, with age and height as independent variables, were derived for males and females in each ethnic group to predict normal pulmonary function for the Singapore Chinese, Malay and Indian populations. The predicted values of various pulmonary function measurements obtained from these regression equations for subjects of specified age (30 years) and height (165 cm for men, 155 cm for women) were compared with those reported in other studies. Differences were observed among the different races.
Fasting serum growth hormone (GH) levels of different groups of diabetic patients were measured and compared to age-matched normal subjects. Insulin-dependent diabetes mellitus (IDDM) children (aged 12-17 years) were found to have significantly lower fasting GH levels than age-matched normal children (p < 0.001). In the adult age groups of 18-44 and 45-76 years, the IDDM patients showed increased fasting GH levels compared to age-matched normal subjects (p < 0.06 and p < 0.001 respectively) and non-insulin-dependent diabetes mellitus (NIDDM) patients (p < 0.05 and p < 0.001 respectively). The fasting GH levels of IDDM patients of the age group 18-44 years also showed significant correlations with glycated haemoglobin (r = 0.510, p = 0.002) and fasting blood sugar levels (r = 0.571, p = 0.01).