Thalassaemia is one of the major public health problems in Malaysia. Regular monthly blood transfusion remains the main treatment for severe thalassaemia patients. One of the complications of blood transfusion is the formation by the recipients of alloantibodies and autoantibodies against red blood cell (RBC) antigen. The purpose of this study was to determine the prevalence of RBC autoantibodies among multiple-transfused thalassaemic patients in our institution and factors that contribute to its development.
Diagnosis and treatment of acute tonsillitis are one of the most common problems seen at an otorhinolaryngology clinic in both adult and paediatric populations. Much has been written about bacteriology of recurrent tonsillitis but it remains a controversial topic. Despite the fact that tonsillitis is so common, consensus seems to be lacking as to the main causative organism and the differences between children and adults. The tonsillar core bacteriology of 233 patients with recurrent tonsillitis who underwent tonsillectomy from January 2000 to June 2003 is presented.
Study site: inpatients, University Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia
Paracetamol is available as an over-the-counter medication in many countries including Malaysia. This drug has been implicated in many poisoning cases admitted to hospitals throughout the country.
To determine the prevalence of iron deficiency (ID) and iron deficiency anaemia (IDA) in Kelantanese pre-school children and to identify risk factors that best predict the presence of ID.
Benign epilepsy of childhood with centrotemporal spikes (BECT) was studied in Malaysian children, and was observed in Chinese, Malay and Indian children in the ratio 10:6:5. There were 12 boys and 9 girls. Fit frequency varied from almost daily to a single fit. The age of onset ranged from 2-13 years and BECT was not noted in any child over 13 years old. There was a strong circadian rhythm and fits occurred mainly in sleep. Generalised seizures were more common than partial seizures. During the 3-year study from April 1989 to April 1992, 21 children with BECT were identified from the EEG records done at the University Hospital and it was found that this genetic epilepsy which is autosomal dominant with age dependent penetrance occurs in approximately 4.8% of our epileptic children. In addition there were 3 children in whom petit mal co-existed with a BECT EEG trait.
Patients on a moderate red cell transfusion programme have iron overload where the concentrations of the serum ferritin were inappropriate to increases in the transfusion load as a result of limitations of apoferritin synthesis and conversion of ferritin into haemosiderin. This study confirms the limitations for the use of estimations of the serum ferritin to evaluate the iron status in patients with expected high overload as would be seen in patients on many years of maintenance red cell transfusions in the absence of iron chelation therapy. Poor compliance, inadequate dosage of Desferal (deferoxamine), and the late initiation of iron chelation therapy were factors that were considered in the patients with failure of response to iron chelation.
A descriptive study of 143 cases of clinically diagnosed measles in patients under the age of 12 years admitted to the Duchess of Kent Hospital, Sandakan, Sabah, during the year 1990 was carried out. The median age of the patients was one year and 13.3% of the cases were between the ages of 6 and 9 months. The male to female ratio was 1.3:1. The majority of the cases (85.3%) were not immunised against measles while 60.0% of the cases were malnourished. Most of the cases (86.0%) had at least one complication with 32.9% of the cases having more than one complications. The main complications were pneumonia (74.1%) and diarrhea (38.5%). Other complications were convulsions, otitis media and corneal ulceration/scarring. A case of pneumonia with mediastinal emphysema and subcutaneous emphysema was noted. The case fatality rate was 1.4% while blindness was the long term morbidity in 1.4% of the cases. Measles remains an important cause of morbidity in children in Sabah.
The overseas Chinese in West Malaysia are almost exclusively from the south-eastern provinces of China-Kwangtung, Fukien, and Kwangsi. To institute a comprehensive thalassaemia control programme for this region we have characterised the beta thalassaemia mutations in 16 Chinese patients from West Malaysia: 4 beta thalassaemia mutations were seen: a) an A----G substitution in the TATA box [-28 base pairs (bp)], an A----T substitution in codon 17 [17 A----T], c) a 4 base pairs - TCTT deletion in codon 41-42 [frameshift mutation (FSC 41-42)], and d) a C----T substitution at the second intervening sequence (IVS 11) position 654. Similar mutations have been described in patients from the south-eastern provinces of China. The delineation of the specific mutations present will enable effective prenatal diagnosis for beta thalassaemia of ethnic Chinese in West Malaysia to be instituted.
The haematological findings and case history of 3 patients with the association of acute myeloid leukemia and translocation involving the long arm of chromosome no. 11 are presented. The recipient chromosome for the translocated material from chromosome 11 differs in all the three cases being namely chromosomes 1, 10 and 17.
We collected data on patients above the age of 5 years with acute bronchial asthma who presented to the emergency room of Hospital Sains Universiti Sains Malaysia during the period between 1 January to 31 March 1990. Two hundred and twelve patients (57% males and 43% females) who made a total of 271 visits were recorded. This constitutes 16.3% of all adults and paediatric medical cases seen in the emergency room during this period. The majority of patients presented between 8 pm and 6 am which contrasts with the attendance pattern due to other causes. We also recorded two peak periods of presentation (between 8 pm and 12 midnight and between 6 am and 10 am). Thirty-one (11.4%) cases resulted in admission. Of the 240 cases that were successfully treated and discharged from the emergency room, there were 59 relapses (in 45 patients). Twenty-two percent of the relapses occurred within 24 hours of the last visits. We recorded lower rates of admission as well as relapses compared to all previous studies. Our finding of bimodal pattern of presentations was also not previously reported.
Study site: Emergency department, Hospital Sains Universiti Sains Malaysia, Kelantan, Malaysia
Three cases of multiple drug resistant falciparum malaria in the same family are described. It is interesting to note that faIciparum malaria resistant to Fansidar has not as yet been reported in adults from West Malaysia up to the present time, although resistance to the drug in children is being encountered not infrequently. This presents a serious paediatric problem because malaria causes the highest incidence of mortality and morbidity in this age group in a proportion of the rural population.
A retrospective study of 224 cases of snake bites in Bukit Mertajam Hospital over a two year period is reported. Snake bites formed 0.5% of the total and 1.7% of the Medical and Paediatric admissions and accounted for 0.3% of the total hospital deaths. The commonest snake species involved was the Malayan pit viper. 89% of the patients escaped with negligible poisoning and went home in less than seven days. Most of the bites were inflicted in the lower limbs and occurred in the dark. Only 5% of the patients were given anti-venom, of whom one-third had adverse reaction.
83 Malays with HbE beta-thalassaemia who were not transfusion dependent were investigated. 79 persons showed no beta0 formation indicating the predominant gene in Malays with HbE beta-thalassaemia was beta0. HbF assays showed levels that were similar to transfusion dependent patients. Further studies are necessary to determine the presence of the alpha, (alpha+) gene Interacting with HbE and beta0 to produce the milder phenotype of HbE beta-thalassaemla.
Plesiomonas shigelloides was isolated from 5 (2.1%) of the 234 children with diarrhoea and none of the 230 controls. In one child, the organism was found in association with Salmonella. Two strains had Shigella sonnei phase I antigen. All the strains were susceptible to the aminoglycosides, cephalosporins, nalidixic acid, nitrofurantoin, chloramphenicol and cotrimoxazole; but resistant to the penicillins. Alkaline peptone water enrichment subcultured to desoxycholale citrate agar proved to be a useful method for isolating this organism from faeces. As the roie of P. shigelloides in causing gastrointestinal disease remains controversial, further studies are necessary to determine its enteropathogenicily.
Hereditary spherocytosis is a rather uncommon disease in Malaysia as only 16 patients were seen in our hospital over a 13 year period. Pallor, jaundice and splenomegaly were common physical signs. Clinical severity of the disease was variable and more than half of them needed splenectomy. Complications including haemolytic crisis and cholelithiasis were encountered but not aplastic crisis. All 10 patients who underwent splenectomy had uniformly good results and none of them had post-operative complications.
Congenital branchial fistula and their embryology is well described in the literature. The accepted standard treatment method includes stepladder excision for extensive fistulas. This paper presents two cases (Case 1 and 2) which are embryologically typical of second and third arch origin respectively; and a third case (Case 3) which has not been previously described. Case 3 appears to be a hitherto unreported combination of first and second arch origin. Case 1 and 2 were treated via a standard stepladder excision approach. A different newer approach was utilised in Case 3, that is, stripping of the fistulous tract with Myer's vein stripper. Unlike the stepladder approach, this is simple and avoids extensive time consuming dissection. However, being a blind procedure, it is not generally advocated if vital structures exist in the proximity.
In this paper the incidence of inhaled foreign body in the tracheobronchial tree at the University Hospital Kuala Lumpur, West Malaysia, is reviewed. Children are the usual victims. Food·related substance (peanut) is the most common foreign body, Successful removal of foreign body by bronchoscopy is achieved in the great majority of cases (86%), Prevention can be achieved by educating the parents not to give nuts to children below the age of 3 years.
115 cases of enucleated eyes in the University Hospital, Kuala Lumpur, are reviewed. Trauma 36, Glaucoma 28, tumours and suspected tumours 24 and Infection 17 are the main causes. Trauma caused mainly by sticks, stones and sharp Instruments Is largely confined to the age group below 30. Primary narrow angle glaucoma accounts for 5 cases. The main types of secondary glaucomas are due to aphakia (12) and a hypermature lens (7) . Two thirds of the tumours are retinoblastomas and this Is the commonest cause of removal below the age of 5 years. There are 4 cases 01 malignant melanoma, all are Chinese. The average age Incidence Is 43 years, which appears to be a decade earlier than in the Caucasians. Preventive measures especially In regards to trauma and glaucoma are discussed.