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Cystic fibrosis (CF) is an autosomal recessive disease commonly found among the Caucasian population. The availability of sweat test and with increasing experience have made it possible to diagnose more cases of CF. Our first case of CF was diagnosed 16 years ago and to date we have managed sixteen cases of CF. Sixteen children were diagnosed with CF in our units at the Paediatric Institute and University Malaya Medical Centre (UMMC). They were referred with either one or all of the following symptoms: i) recurrent pneumonia, ii) bronchiectasis, iii) failure to thrive, iii) malabsorption or iv) history of meconium ileus obstruction during the neonatal period. When the clinical features suggested strongly of CF, sweat tests will be performed in duplicates and considered positive when the sweat chloride or sweat sodium was more than 60 mmol/l for both results. Seventy- two hours fecal fat excretion or stool for fat globule was performed to document malabsorption. From the year 1987 to 2003, 16 patients were confirmed to have cystic fibrosis in Malaysia by positive sweat tests. Thirteen patients were diagnosed in Paediatric Institute while the remaining three were diagnosed in UMMC. On follow-up two patients died due to severe bronchopneumonia at the age of two years old. Although once considered rare, CF should now be considered in any children with clinical presentations of recurrent chest infections, bronchiectasis, in the presence or absence of malabsoption stmptoms and in neonates with meconium ileus obstruction.

Unlabelled: Respiratory syncytial virus (RSV) chest infection is a common cause of hospitalization in the very young child. The aim of this study was to determine the direct cost of resource utilization in the treatment of children hospitalized with RSV chest infection and the potential cost-savings with passive immunization for high-risk infants. An audit of the hospital resource consumption and its costs was performed for 216 children aged < 24 mo admitted with RSV chest infection between 1995 and 1997. The cost-saving potential of passive immunization using monoclonal RSV antibodies during the RSV season was determined by assuming an 0.55 efficacy in hospitalization reduction when administered to "high-risk" infants according to the guidelines outlined by the American Academy of Pediatrics (AAP). The hospital treatment cost of 1064 bed-days amounted to USD 64 277.70. Each child occupied a median of 4.0 bed-days at a median cost of USD 169.99 (IQ1 128.08, IQ3 248.47). Children, who were ex-premature or with an underlying illness were more likely to have a longer hospital stay, higher treatment costs and need for intensive care. Ten (42%) of 24 ex-premature infants fulfilled the recommended criteria for passive immunization. Its use resulted in an incremental cost of USD 31.39 to a potential cost saving of USD 0.91 per infant for each hospital day saved., Conclusion: Ex-prematurity and the presence of an underlying illness results in escalation of the direct treatment cost of RSV chest infection. Current guidelines for use of passive RSV immunization do not appear to be cost-effective if adopted for Malaysian infants.

Accessibility of research done locally to clinicians remains limited unless it is in the published form. The publication rate of research presentations at the Annual Malaysian Paediatric Association, Perinatal Society of Malaysia and Academy of Medicine Malaysia in 1997 and 1998 was determined. One hundred and five (95.5%) of 110 research presentations were carried out in Malaysia. Thirty-seven (35.2%) presentations were published. University-affiliated institutions were more likely to publish their research presentations as compared to Ministry of Health hospitals (OR 3.1 95% CI 1.4-6.8, p < 0.01). There is a need to encourage publication of local research presentations. University-affiliated institutions performed better due to institution pressure for career advancement.

A pilot study to evaluate the direct cost of treating 51 adults and 50 children with bronchial asthma was conducted. All aspects of the medical care provided over a 6-month period were considered. The mean treatment costs per month were US dollars 22.97 (adults) and US dollars 15.56 (children). The cost of maintenance therapy accounted for 55.5% and 73.4% of the total direct cost treatment for adults and children respectively. Only 27 (52.9%) adults and 17 (34.0%) children paid for their inhaled prophylactic drugs, amounting to 12.3% of the total maintenance therapy costs. Thirteen (25.4%) adults and 9 (18.0%) children were using alternative therapy at a monthly cost of US dollars 41.50 and US dollars 16.77 respectively. A substantial proportion of the direct cost of asthma treatment is heavily subsidized in Malaysia. Adequate attention to the allocation of the health budget, to ensure the optimal provision of health care, is warranted.

Respiratory syncytial virus (RSV) is the most important cause of lower respiratory tract infection (LRTI) in young children. We determined if there was a seasonal variation in Malaysia in the incidence of RSV infection in young children admitted with LRTI, and possible associations of RSV infection with local meteorological parameters. A total of 5,691 children, aged less than 24 months and hospitalized with LRTI (i.e., bronchiolitis and pneumonia) between 1982-1997, were included in this study. Nasopharyngeal samples were collected and examined for RSV by immunofluorescence, viral culture, or both. Seasonal variations were determined by analyzing the monthly RSV-positive isolation rate via time series analysis. Possible correlations with local meteorological parameters were also evaluated.RSV was isolated in 1,047 (18.4%) children. Seasonal variations in RSV infection rate were evident and peaked during the months of November, December, and January (test statistics [T] = 53.7, P < 0.001). This seasonal variation was evident for both bronchiolitis and pneumonia categories (T = 42.8 and 56.9, respectively, P < 0.001). The rate of RSV infection appeared to correlate with the monthly number of rain days (r = 0.26, P < 0.01), and inversely with the monthly mean temperature (r = -0.38, P < 0.001). In the tropics, seasonal variations in the incidence of RSV infection are evident, with an annual peak in November, December, and January. This information provides a guide for healthcare provisions and implementation of RSV prevention., (Copyright 2002 Wiley-Liss, Inc.)

Objective: To determine the risk factors associated with severe viral croup in hospitalised Malaysian children., Methodology: The medical records of children aged less than five years admitted with a diagnosis of viral croup between 1994 and 1999 were reviewed. Severe viral croup was diagnosed in children who had stridor at rest with marked recession associated with central cyanosis or altered level of consciousness. Multivariate logistic regression analysis was performed to identify risk factors associated with severe viral croup., Results: Eighteen (14.7%) of 122 children with viral croup were severe. These children were older (mean age 16.8 +/- 7.2 vs 12.6 +/- 6.6 months, p = 0.01) and had a shorter duration of illness prior to admission (1.7 +/- 0.7 days vs 2.3 +/- 1.4 days, p = 0.03). Age between 12 and 24 months (OR 3.8 95% Cl 1.3, 12.7, p = 0.02) and fever (OR 5.7 95% Cl 2.9, 15.6, p = 0.02) were the only risk factors associated with severe viral croup after multivariate logistic regression analysis. Only three children or 2.5 per 100 children admitted with viral croup required ventilation., Conclusion: Only a small number of children admitted particularly those between 12 to 24 months with fever developed severe viral croup. Recognition of these risk factors provides a guide in selecting children who will most likely benefit from steroid therapy. The overall outcome was nonetheless favourable.

Background: The larva of Toxocara spp., a common animal roundworm, may infect non-compatible hosts, causing a profound immunological reaction with marked eosinophil and IgE responses, not unlike in atopy. In this study, we determined the seroprevalence of Toxocara exposure in 66 asthmatic and 58 non-asthmatic children., Methods: Exposure to Toxocara was determined by examining the serum samples of the children for specific IgG antibodies to L2 Toxocara larvae, using a commercially available diagnostic kit., Results: There was no significant difference in the mean age, sex, social class, residence type and presence of domestic pets at home between the two children groups. Children with bronchial asthma were observed to have higher Toxocara seropositivity than that of the non-asthmatic controls (21.2 vs 8.6%, P=0.047)., Conclusion: The observed relationship between exposure to Toxocara infection and bronchial asthma in Malaysian children warrants further evaluation. An understanding of any possible contribution to the pathogenesis of childhood asthma provides a potential avenue for prevention.

Mycoplasma pneumoniae is increasingly recognized as an important cause of community acquired pneumonia (CAP) in children. We determined the importance of M. pneumoniae as a causative agent in 170 children aged 1 month to 15 years who were hospitalized with CAP over a 6-month period. The diagnosis of M. pneumoniae infection was based on serological evidence obtained by a particle agglutination test (SERODIA-MYCO II). A positive serological diagnosis was made if the acute phase serum titer was more than 1:160 or paired samples taken 2-4 weeks apart showed a four-fold or greater rise in the serum titer. M. pneumoniae was identified as the causative agent in 40 (23.5%) children. Children with M. pneumoniae infection were more likely to be older than 3 years (OR 4.0 95%CI 1.8-9.1, p<0.001), Chinese (OR 4.3 95%CI 2.0-8.9, p<0.001), have a duration of illness longer than 7 days prior to admission (OR 6.0 95%CI 2.7-13.5, p<0.001) and have perihilar interstitial changes on chest X-ray (OR 4.6 95%CI 2.2-9.9, p<0.001). A significant number of hospital admissions for CAP in Malaysian children can be attributed to M. pneumoniae. It is important to identify these children so as to administer the most appropriate antibiotic treatment.

Background: The clinical profile of severe upper airway obstruction, a challenging acute pediatric emergency, has not been extensively documented in the developing nations of the tropics., Methods: The diagnostic categories, severity of illness and outcome from 63 episodes of severe upper airway obstruction in 56 children admitted to the Pediatric Intensive Care Unit between January 1994 and December 1999 were reviewed. Outcome variables studied included requirement for ventilation, mortality and complications. Severity of illness was determined with the Pediatric Risk of Mortality (PRISM) II score., Results: Viral croup (29%) was the most common diagnosis, followed by mediastinal malignancy (13%), bacterial tracheitis (11%) and Pierre Robin syndrome (11%). There were no admissions for acute epiglottitis. Thirty episodes (48%) required ventilation for a median duration of 4.0 days. Bacterial tracheitis (100%) and subglottic stenosis (100%) were the most likely diagnoses requiring ventilation. Difficulty in intubation was encountered in 13 episodes (43%) involving, in particular, patients with bacterial tracheitis (83%; P = 0.006). Only two patients required a tracheostomy. The overall mortality was 11%. The PRISM score for all categories was generally low (mean 10.3 +/- 1.0; median 9.0). Non-survivors had a significantly higher PRISM II score than survivors (27.4 +/- 9.7 vs 8.1 +/- 4.9, respectively; P = 0.002) and were more likely to include children with bacterial tracheitis and mediastinal malignancy., Conclusions: There is marked heterogeneity in the causes of upper airway obstruction in the tropics with viral croup remaining the most common. A significant proportion required ventilation, but outcome is generally favorable, except in those with bacterial tracheitis and mediastinal malignancy.

Blood pressure examination was done manually in 1756 healthy school children aged 6-12 years. Korotkoff 1 represented the systolic blood pressure (SBP) and Korotkoff 5 was taken as the diastolic blood pressure (DBP). Blood pressure percentile charts were then drawn up based on age group and sex regardless of ethnicity. There was a significant correlation between both SBP and DBP to increasing height, weight and body mass index.

Twenty-two (42 per cent) out of 52 patients admitted with severe bronchiolitis to our Paediatric Intensive Care Unit required ventilation. Risk factors associated with ventilation included a younger mean age, female sex, low birthweight, failure to thrive and the presence of an underlying illness. Ventilated patients were also more likely to have a higher respiratory distress assessment index (RDAI) score, pneumonic infiltration on chest X-ray, lower serum sodium and a positive respiratory syncytial virus isolation in the tracheal secretion.

Paediatric intensive care in Malaysia is a developing subspecialty with an increasing number of specialists with a paediatric background being involved in the care of critically ill children. A part prospective and part retrospective review of 118 consecutive non-neonatal ventilated patients in University Hospital, Kuala Lumpur was carried out from 1 June 1995 to 31 December 1996 to study the clinical epidemiology and outcome in our paediatric intensive case unit (PICU). The mean age of the patients was 33.9 +/- 6.0 months (median 16 months). The main mode of admission was emergency (96.6 per cent) with an overall mortality rate of 42 per cent (50/118). The mean paediatric risk of mortality (PRISM) score was 20 +/- 0.98 SEM, with 53 per cent of patients having a score of over 30 per cent. Multiorgan dysfunction (MODS) was identified in 71 per cent of patients. Admission efficiency (mortality risk > 1 per cent) was 97 per cent. Standardized mortality rate using PRISM was an acceptable 1.06. The main diagnostic categories were respiratory (32 per cent), neurology (22 per cent), haematology-oncology (18 per cent); the aetiology of dysfunction was mainly infective. Non-survivors were older (29.5 vs. 13.8 months, p < 0.0001), had more severe illness (mean PRISM score 30 vs. 14, p < 0.0001), were more likely to develop MODS (96 vs. 53 per cent, p < 0.0001) and required more intervention and monitoring. Paediatric intensive care in Malaysia differs widely from that in developed countries in patient characteristics, severity of illness, and care modalities provided.

Objective: To study the viral aetiology of lower respiratory tract infection (LRTI) in young Malaysian children., Methodology: A retrospective review was performed of LRTI patients aged less than 24 months who were admitted to the University Malaya Medical Centre between 1982 and 1997. Respiratory viruses in their nasopharyngeal secretion were identified by indirect immunofluorescence, viral culture, or both., Results: A total of 5691 children were included in the study. The mean age was 8.6 +/- 6.6 months and the M:F ratio was 1.6:1. The most common diagnosis was pneumonia (52%) followed by bronchiolitis (45%) and croup (2%). Positive viral isolation rate was 22.0%. Respiratory syncytial virus (RSV) was the commonest virus isolated (84%), followed by parainfluenza virus (8%), influenza virus (6%) and adenovirus (2%). Patients with positive virus isolation were younger (7.8 +/- 6.2 vs 8.7 +/- 6.7 months, P = 0.0001) and were more likely to have bronchiolitis., Conclusion: Young Malaysian children admitted with LRTI had a 22% viral isolation rate and RSV was the commonest virus isolated.

Objectives: To compare the modes of death and factors leading to withdrawal or limitation of life support in a paediatric intensive care unit (PICU) in a developing country., Methods: Retrospective analysis of all children (< 12 years) dying in the PICU from January 1995 to December 1995 and January 1997 to June 1998 (n = 148)., Results: The main mode of death was by limitation of treatment in 68 of 148 patients, failure of active treatment including cardiopulmonary resuscitation in 61, brain death in 12, and withdrawal of life support with removal of endotracheal tube in seven. There was no significant variation in the proportion of limitation of treatment, failure of active treatment, and brain death between the two periods; however, there was an increase in withdrawal of life support from 0% in 1995 to 8% in 1997-98. Justification for limitation was based predominantly on expectation of imminent death (71 of 75). Ethnic variability was noted among the 14 of 21 patients who refused withdrawal. Discussions for care restrictions were initiated almost exclusively by paediatricians (70 of 75). Diagnostic uncertainty (36% v 4.6%) and presentation as an acute illness were associated with the use of active treatment., Conclusions: Limitation of treatment is the most common mode of death in a developing country's PICU and active withdrawal is still not widely practised. Paediatricians in developing countries are becoming more proactive in managing death and dying but have to consider sociocultural and religious factors when making such decisions.

Objectives: To determine the incidence and outcome of acute respiratory distress syndrome (ARDS) in children by comparing two commonly used definitions: the lung injury score and the American-European Consensus Conference definition. The causes and risk for developing ARDS were also studied., Methods: Part prospective and retrospective analysis of 8100 consecutive hospital admissions from 1 June 1995 to 1 April 1997., Results: Twenty one patients fulfilled the criteria for ARDS. Both definitions identified the same group of patients. The incidence was 2.8/1000 hospital admissions or 4.2% of paediatric intensive care unit admissions. The main causes were sepsis and pneumonia. Mortality was 13 of 21. Factors predicting death were a high admission paediatric risk of mortality (PRISM) score (30.38 v 18.75) and the presence of multiple organ dysfunction syndrome (92% v 25%)., Conclusion: Both definitions identified similar groups of patients. The incidence in this population was higher than that reported elsewhere, but mortality and cause were similar to those in developed countries. Poor outcome was associated with sepsis, a high admission PRISM score, and simultaneous occurrence of other organ dysfunction.