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.
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).
The aim of this study was to investigate if colloidal bismuth subcitrate (CBS) can penetrate the gastric mucus barrier to reach the different sites of the antral mucosa and to estimate the time course for CBS to reach and remain in the mucosa. A single dose of CBS was administered orally to rats that were sacrificed at different time intervals post treatment. The control group received gum acacia without CBS. Colloidal bismuth subcitrate, visualised as electron dense precipitate (EDP), was seen in the gastric mucus layer, intercellular spaces and intracellularly after 30 minutes and disappeared after 6 hours. Scant amounts of EDP were observed in the gastric crypts, confined only to the upper parts of these structures. We concluded that CBS can penetrate the mucus and has a wide but uneven distribution in the gastric mucosa. Colloidal bismuth subcitrate, in the concentration given only penetrated the upper two-thirds of gastric pits and not the lower one-third. We also concluded that CBS has to be given 6 hourly to ensure its continuous presence in the gastric mucosa.
Fresh frozen neoplastic tissues from 70 infiltrating ductal breast carcinomas were analysed for cytosolic oestrogen receptor (ER) protein content using a solid phase enzyme immunoassay (EIA) method based on a "sandwich" principle (Abbott ER-EIA monoclonal). Formalin-fixed, paraffin-embedded sections from the same carcinomas were examined for nuclear immunoreactivity against a monoclonal antibody for ER protein (Dako) using the standard avidin-biotin complex immunoperoxidase (IP) method after microwave antigen retrieval. The degree of ER positivity by IP was also scored according to a visual estimation of the percentage of cells expressing immunopositivity and the intensity of staining. Twenty-eight (40%) of the carcinomas were ER-positive by EIA and 34 (48.6%) were positive by IP. Twenty-five (35.7%) were ER-positive and 33 (47.1%) were ER-negative by both methods. Nine (12.9%) were ER-negative by EIA but were positive by IP, this discrepancy being ascribed to sampling inadequacy for EIA. However, 3 (4.3%) tumours were ER-positive by EIA and negative by IP. This discrepancy may be variously due to inadequate antigen retrieval, faulty technique and the possibility that the two methods do not measure identical ER proteins. IP appears to have an advantage over EIA in that it has a higher pick-up rate, does not require fresh tissue and can be applied to archival material. However, to reduce false negative estimations, it may be necessary to run IP staining using more than one ER antibody. Standardisation of the IP method for ER is desirable before this method is to be widely adopted in Malaysian laboratories. Quantitation of ER positivity by IP scoring correlated poorly with actual cytosolic levels. Caution should be exercised in attaching patient management value to visual IP scoring.
Sixty-one free flaps performed in 59 patients from April 1983 to April 1995 were analysed. Various factors that might have affected the outcome of the surgery were studied. These included the patient's age, history of smoking, pre-existing medical problems such as hypertension and diabetes mellitus, the type of free flaps, flap infection, use of postoperative anticoagulation, postoperative anaemia and re-exploration. The infection rate was 16.4% and this had a strong correlation with the free flap failure in our study population. Postoperative anaemia could adversely affect the tissue oxygenation of the free flap and delay the re-exploration due to the high anaesthetic risk. Dextran was routinely used for postoperative anticoagulation. There were also rescue attempts using heparin infusion when needed. The overall failure rate was 13.1%. Besides good anaesthetic support, a well-prepared protocol is necessary both for the preoperative planning of free flap surgery as well as salvaging a failure.
Since dengue was first documented in Malaysia in 1902 and made notifiable in 1973, the disease pattern has changed from major outbreaks every four years to one of increasing trend yearly. The largest outbreak was seen in 1996 with 14,255 dengue cases reported and 32 deaths. The case fatality rate varied from a high of 10.43% in 1985 when dengue type 3 was the predominant type to a low of 1.29% when dengue type 1 predominated. Severe disease patterns have been observed with dengue 2 and 3 serotypes in the country. The clinical spectrum has also been changing and multisystem involvement with more severe manifestations are being seen. Liver involvement has been documented since 1987. Fulminant hepatitis with encephalopathy can resemble Reye's syndrome. Dengue type 3 has been isolated from liver biopsy specimens. Neurological manifestations can very from irritability, convulsions, coma to peripheral neuritis. The isolation of dengue viruses from cerebrospinal fluids recently strongly suggests that dengue viruses can be neurovirulent. Adult respiratory distress syndrome was seen in three children admitted with shock. Deaths were more frequent in children in the early period but since 1982, over 50% of deaths have occurred in patients over the age of 15 years.
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).
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).
To assess the utility of the current imaging modalities, a six-year retrospective study was conducted from May 1985 through April 1991 on 11 patients with histopathologically confirmed neuroblastoma. There were four males and seven females. Median age at presentation was six years. All patients underwent intravenous urography (IVU), abdominal ultrasonography (US), skeletal surveys and bone marrow aspiration. Computed tomographic (CT) scanning was performed in nine patients and trephine bone biopsy in five. Suprarenal gland was the primary site in six patients, including one bilateral neuroblastoma. Two presented with primary paraspinal mass, one with posterior mediastinal mass, one had a retroperitoneal tumour and one presented as primary cerebral neuroblastoma. IVU showed indirect evidence of suprarenal tumours in five patients. US demonstrated suprarenal mass in six and calcification within the mass in five patients. US also documented retroperitoneal lymphadenopathy in five patients. US-guided fine needle aspiration biopsy was performed in seven patients and it gave correct histological diagnosis in all. CT accurately depicted all the primary tumours and their involvement of the surrounding structures and metastases in the brain and orbits. US proved to be quite accurate in diagnosing primary abdominal tumours despite its limitations but in other sites, CT was of greater help. Solid appearance of a suprarenal mass on US made the diagnosis of neuroblastoma very likely and calcification within the mass further supported the diagnosis.
A bank explosion in a neighbouring country over 1000 km away resulted in ten badly burned victims being airlifted to the Burns Centre, Singapore General Hospital (BCSGH) for treatment. The severely injured included patients with 90%, 80%, 74%, 66%, 45%, 33% and 31% burns. Nine had respiratory burns (four severe, one moderate, four mild). One patient died, thus, the mortality rate for the six most severely injured was 16.7%. This differs from predicted mortality rates of 78% according to McCoy or 54% according to Thompson, Herndon et al. The factors contributing to this result were the small size of the disaster, the use of an established Burns Mass Disaster plan and an individual management policy that incorporates carefully monitored fluid resuscitation, recognition of respiratory burns with early treatment by intubation thus pre emptying complications, early surgery and a multidisciplinary approach to complications such as infection and renal failure. The average length of stay was 43 days (range 5-122 days). The cost of the hospitalisation of the ten casualties was $312,317.00.
In Asian countries, it is more difficult to obtain cadaver kidneys for renal transplantation because of certain socio-cultural beliefs and customs. The issues affecting living related kidney donation are more social than cultural. This is due to the web of family pressures and personal conflicts for both donor and recipient surrounding the donation. Important misconceptions and fears are: fear of death, the belief that removal of organ violates sanctity of decreased, concern about being cut up after death, desire to be buried whole, dislike of idea of kidneys inside another person, wrong concept of brain death, and the idea of donation being against religious conviction. In Singapore, with the introduction of the Human Organ Transplant Act (HOTA) in 1988, the number of cadaveric transplants have increased, including those from the Medical Therapy Act (MTA). HOTA and education have played pivotal roles in bringing about an increased yield of cadaveric kidneys. With the availability of living unrelated donor (LUD) transplants in India, our living related donor (LRD) transplant programme has suffered, because patients would rather buy a kidney from overseas than get a relative to donate one. Patients are also going to China for overseas cadaveric transplants where the kidneys come from executed convicts. People in countries like Hong Kong, Japan and the Philippines share the same Asian tradition of not parting with their organs after death. Muslim countries like Malaysia require the deceased to have earlier pledged his kidneys for donation prior to death before they can be harvested for transplantation at death.
Most muscular dystrophies manifest as peripheral muscular weakness commencing at various age, however, oculopharyngeal muscular dystrophy (OPMD) is a rare hereditary disorder presenting in middle age with progressive dysphagia and bilateral blepharoptosis rather than peripheral muscular weakness. In the medical literature, OPMD has been well described in Canadians of French descent. So far, there is no publication of OPMD in the Malaysia-Singapore medical literature. This article documents this condition in a Chinese patient. A review of the literature is presented and the management of OPMD is discussed.
The aim of this retrospective study was to characterise the clinical presentation and disease associations of Oriental patients with gout seen in our hospital over a six-month period. One hundred patients comprising of 77 males and 23 females [89% Chinese, 7% Malays, 2% Indians and 2% others; mean age was 50.9 years (range 18 to 82 years), mean age at onset of disease was 43.7 years (range 16 to 78 years)] were studied. The disease was familial in 18% and 44% of patients had a history of alcohol ingestion. Co-morbid conditions included hypertension (36%), hyperlipidaemia (25%), renal failure (17%), ischaemic heart disease (13%), diabetes mellitus (4%), systemic lupus erythematosus (3%), psoriasis (2%) and ankylosing spondylitis (1%). The majority of patients (68%) had at least one associated disease. At the onset of disease, the joints commonly involved were the ankles (39%) and knees (27%) whilst the first metatarsophalangeal (MTP) joint was affected in only 26% of cases. Polyarticular onset was uncommon (n = 6). The precipitating factors reported by the patients included food (n = 23), alcohol (n = 12), drugs (n = 4), trauma (n = 3) and surgery (n = 2). Eleven patients had a history of renal calculi and 15% had tophaceous gout. Majority of patients (71%) had been treated with urate-lowering drugs (allopurinol). We concluded that gout in Singapore predominantly affects middle-aged men who often have an accompanying illness.
This paper presents the results of a clinical study of 150 patients in Singapore with ankylosing spondylitis (AS) and reviews recent developments locally with regards to the disease. The patients were predominantly males (ratio 7:1) and Chinese (n = 147). The onset of disease is usually in the early twenties and there was a mean delay of 6.3 years before diagnosis was made. Peripheral joint involvement is common but apart from uveitis (17%), extra-articular manifestations are rare. AS patients have abnormal lipid profiles and lower bone mineral density compared to healthy controls. HLA*B2704 is the predominant subtype in our Chinese patients whilst HLA*B2706 was found only in healthy controls. Intensive group physiotherapy is beneficial for patients with spondyloarthropathy.