Dengue haemorrhagic fever (DHF) is a severe viral illness with significant morbidity and mortality especially among children in Southeast Asia. The tourniquet test is recommended by the World Health Organisation (WHO) as an initial clinical screening procedure for patients suspected to have DHF, particularly in patients with DHF grade I. The aim of this study was to evaluate the validity of the tourniquet test as a predictor of DHF and also to assess the usefulness of repeated, serial tourniquet testing as a diagnostic indicator of DHF. Individuals included into this study were children from the Institute of Paediatrics, Kuala Lumpur who were admitted on a clinical suspicion of DHF based on the WHO criteria and who had serology for Dengue IgM performed. A standard method of tourniquet was performed on these patients on a daily basis following admission. A total number of 60 patients were considered for analysis and of these the tourniquet test was positive in 50 patients and negative in the remaining 10 patients. Sensitivity of the test was found to be 85.4% and the specificity was 25%. Further assessment of the predictive value of the test showed that the positive predictive value (PPV) was 82% while the negative predictive value (NPV) was 30%. In conclusion, a positive tourniquet test, serially performed on a daily basis was found clinically to be a useful preliminary screening tool for DHF as recommended by WHO. However its specificity was low and consequently led to a high false positive rate.
A retrospective study was conducted to evaluate the magnitude, clinical course and risk factors of cholestasis in surviving very low birth weight (VLBW) infants who received parenteral nutrition at the neonatal intensive care unit (NICU) of Hospital Universiti Kebangsaan Malaysia. Cholestasis was defined as direct hyperbilirubinaemia of >34 mmol/L. Between 1St July 2000 to 31St March 2001, 58 VLBW infants received parenteral nutrition (PN). Forty seven infants survived to discharge. Complete data was obtained from 43 (90.1%) infants. Thirteen (30.2%) of these infants developed cholestasis. The cholestasis persisted beyond the age of 6 weeks in 8 infants and they underwent hepato imino diacetic acid (HIDA) scan. The HIDA scan was reported as normal in 2 infants and inconclusive in 6 infants. Operative cholangiography. (OTC) was then performed in these infants and were all normal. The cholestasis finally resolved in all infants. Analysis of risk factors revealed that duration of assisted ventilation and PN and the presence of patent ductus arteriosus (PDA) had significant correlation with the development of cholestasis. These factors together with lower mean gestational age increased the likelihood of persistent cholestasis beyond 6 weeks of life in these infants. In conclusion cholestasis is common in VLBW infants who received parenteral nutrition. Although the clinical course seems benign, in a significant proportion of these infants it may persist longer and put them at increased risk of requiring invasive investigations.
Objectives: 1) To establish the incidence of rotavirus infection in paediatric patients admitted to a private hospital in Malaysia with a diagnosis of community-acquired acute gastroenteritis. 2) To determine whether patients with rotaviral acute gastroenteritis have greater disease severity. Design Retrospective cross-sectional study. Setting An urban private hospital in Malaysia. Participants All paediatric patients with a discharge diagnosis of acute gastroenteritis (AGE) admitted to the hospital between September 2005 and March 2006. A total of 261 patients were included in the study. Results Rotavirus infection contributed to 54% of paediatric hospital admissions with discharge diagnoses of acute gastroenteritis. 74% of children with rotavirus infection were aged 5 years and below. This study demonstrated that patients with rotaviral gastroenteritis had greater disease severity, as measured by two indicators. Firstly, the average length of hospital stay was longer in patients who were rotavirus positive (5.14 days) compared to those who were rotavirus negative (4.81 days). These results were statistically significant (p
Spinal Muscular Atrophy (SMA) is a heredity neuromuscular disorder and is one of the most common genetic causes of childhood fatality. SMA is classified into three groups based on age of onset and achieved motor milestone. Survival Motor Neuron (SMN) gene has been identified as the responsible gene for SMA. From August 2003 until Feb 2007 we have received 93 samples for SMN1 gene deletion analysis from various hospitals in Malaysia. All the patients except for 3 patients were Malaysian (71 Malays, 5 Indians, 9 Chinese and 5 patients are mixed ethnicity). DNA were extracted from blood samples using DNA extraction kit and subjected to SMN/ gene deletion analysis by PCR-RE. Forty nine out of 93 samples (20 type I, 21 type II, and 8 type III) were found to have homozygous deletion of at least exon 7 of the SMN1 gene. Twelve patients (7 type I, 4 type II, 1 type III) showed the presence of the SMN1 gene and the rest were excluded as they did not fulfill the criteria of International SMA Consortium. Deletion analysis of exon 7 of the SMN gene can be an alternative to the existing diagnostic modalities of SMA.
Background: Kawasaki Disease (KD) and acute rheumatic fever are the two leading causes of acquired heart disease in children in the developing countries. Objectives: To determine the epidemiology of KD and its short-term outcome in Malaysian children. Materials & Methods: A retrospective study of patients with a diagnosis of KD at the Kuala Lumpur Hospital from January 1999 to December 2003. Results: 84 patients with KD were seen over the 5 year period. Of these, 52 (61.90%) were male and 32 (38.10%) female. Malays comprised 51 (60.71%), Chinese 30 (35.71%) and Indian 3 (3.57%). Their ages ranged from 2 months to 11 years 1 month old. There were 25 (29.76%) patients less than one year old, 50 (59.52%) aged 1-4 years, 4 (4.76%) aged 5-7 years and 5 (5.95%) were more than 7 years old. Echocardiographic examination during the acute phase showed that 24 patients (28.57%) had coronary artery dilatation; 23 had mild dilatation and one had giant aneurysm involving both coronary arteries. Echocardiographic examination at 8 weeks showed that 5 (21.74%) of the 23 patients with mildly dilated coronary arteries had resolved; the bilateral giant coronary aneurysms remained the same. There was no death due to KD over the 5 year period. Conclusion: KD occurred most commonly in children aged 1-4 years old with a peak at 17 months. There was male preponderance with a male to female ratio of 1.6:1. Despite immunoglobulin therapy, 29% of patients had coronary artery involvementduring the acute phase; 22% of those with mild coronary artery dilatation resolved at 8 weeks after disease onset.
Respiratory papillomatosis is a disease of viral origin which is characterized by warty exophytic lesions in the aerodigestive tract. It is the most common benign lesion of larynx and the second most common cause of hoarseness in children. It has the tendency to recur and to spread through out the entire length of the aerodigestive tract. Although a benign disease, it has the potential of morbid consequences in view of airway complications and the risk of malignant transformation. We report a case of juvenile onset of respiratory papillomatosis and its therapeutic challenges.
Introduction: There are many factors that determine the survival of patients with VSD. Among these include size of VSD, position, pulmonary hypertension, bacterial sepsis, valvular involvement, associated anomalies with VSD, associated syndromes and age at first diagnosis. There has been no published local data as far as we know and this information will be useful especially for consultation with parents. Even though VSD in general has a good prognosis, whenever they have added risks for example pulmonary hypertension then they are at risk of further morbidity and mortality. Objective: To determine the factors that are associated with survival of patients with VSD. Design: Retrospective cohort. Materials and methods: All cases of isolated VSDs admitted to HUSM from 1996 to 2003 were reviewed. Results: Univariate Cox regression of survival time of patients with VSDs revealed that 4 factors had prognostic significance namely bacterial sepsis (HR= 287.7, 95% CI 51.1, 1618.5, P < 0.001), Down syndrome (HR = 14.89, 95% CI 3.00, 73.92, P = 0.001), pulmonary hypertension (HR=14.58, 95% CI 1.69, 125.7, P=0.015) and large VSDs (HR=8.23, 95% CI 1.5, 45, P=0.015). Bacterial sepsis was the only significant prognostic factors for the survival of patients with VSDs using the multivariate Cox proportional hazard model. Conclusion: Bacterial sepsis, pulmonary hypertension, large VSD and Down syndrome were the significant prognostic factors from Univariate Cox analysis, however bacterial sepsis was the only significant prognostic factor from Multivariate Cox analysis.
Respiratory syncytial virus (RSV) is isolated in 15-25% of young Malaysian children with bronchiolitis.',2 Although this observation is consistent with experience reported in other developing nations in the tropics,3•4 it is lower than that of temperate developed nations where RSV is isolated in 60 - 80% of young children with viral bronchiolitis.5,6 The majority of infections are mild, easily cared for at home and only 1% of children with RSV bronchiolitis require in-hospital care.' However, several categories of children have been identified to develop severe RSV bronchiolitis that is asso-ciated with an increased risk of mortality and significant morbidity. This 'high-risk' group includes children who are very young, ex-premature (gestation less than 36 weeks), children with chronic lung disease, congenital heart disease and immunodeficiency, namely, haematological transplant recipients.8'9 It is for this category of children in whom effective therapeutic strategies for the treatment of RSV bronchiolitis are most important.
The medical education should be tailored to deal with the diseases the physician is most likely to see.' With expectations that all the graduates from Malaysian Medical Schools should be able to serve anywhere in the country, the need for a national curriculum is self-evident. It may be argued that the public must have confidence in the competence of the practitioners they depend upon irrespective of the school from which they had graduated. In smaller countries in which health needs are uniform the graduates of any school should have been trained to meet those needs. In larger countries and those with geographic diversities and distances (e.g. Malaysia), the curriculum should cover the commonly encountered diverse ailments. If not dealt with care, we may end up with huge load of ever expanding, unmanageable curriculum.
Life (QOL) of patients with childhood leukaemias presenting at the Hospital Universiti Kebangsaan Malaysia (HUKM). The objectives of this pilot study were 1) To assess the feasibility and applicability of assessing quality of life in leukaemia patients using the adult-based SF-36 questionnaire. 2) To compare the differences of QOL scores among patients based on gender and treatment status. The Short-Form-36 Health Survey (SF36) was used. The items in SF-36 were drawn from the original 245-item MOS questionnaire, which includes multi-item scales that measured the eight dimensions of quality of life namely physical function, role limitations (physical problems), bodily pain, general health, vitality, social functioning, role limitations (emotional problems) and mental health. Patients with acute leukaemias were chosen to participate in the study. Twenty-eight patients were recruited, twelve were males and 16 were females. Ten patients had completed therapy whilst 18 others were still undergoing treatment. The results showed that those patients off treatment have a better quality of life than those on chemotherapy. However, this was only significant with respect to the role limitation pertaining to physical problems. Although the use of the SF-36 was feasible, there were limitations especially in the younger age group.
Forty thousand children die each day in developing countries; which is approximately 28 dead children every minute. In many of these countries 25% of their children do not even live long enough to celebrate their fifth birthday. These mortality statistics represents a human tragedy affecting our very youngest. The United Nations Children's Fund (UNICEF) has identified seven simple, practical and cost-effective activities to help save the lives of our children. The acronym GOBI FFF - Child Survival Strategy describes these 7 measures. The B in the acronym represents beastfeeding which is a pivotal and fundamental component of this child survival strategy.
Generally, the karyotype profile of Down Syndrome has been reported to be full trisomy 21 in 92% of patients, mosaic trisomy 21 in 4% of patients and translocation involving chromosome 21 in 4% of patients in most of the population groups worldwide. But, karyotype analysis of 149 DS patients at the Human Genome Center, USM, during the past five years revealed that free trisomy accounted for 94.6%, mosaic trisomy 21 for 4.7% and translocation involving chromosome 21 in 0.7% of the Down Syndrome etiology in North East Malaysian population, indicating a low frequency of translocation DS in this region. Here, we report one case of translocation Down Syndrome encountered during karyotype analysis of 149 DS cases. Karyotype showed a robertsonian translocation where an entire extra chromosome 21 was attached to the centromere of one of the chromosome 14, resulting in a derivative chromosome 14 with attached chromosome 21. Karyotype analysis of the parents revealed a normal 46,XY pattern for father and 46,XX pattern for mother indicating that this robertsonian translocation had arisen de novo either prior to or at conception.
Introduction: Parents play an important role in the management of their asthmatic children. Thus the ability of parents to recognise asthma trigger factors are very important.
Objectives: The objectives of this study were to identify the trigger factors that were recognised by parents to cause acute exacerbation in their children and analyse the association of these factors with severity of asthma and parental asthma knowledge.
Methods: Sixty-seven parents were interviewed to identify factors that can exacerbate acute asthmatic attack in their asthmatic children. The factors were then categorised as: infection, exercise, allergen, irritant, emotion and weather. The profiles of children were asthma severity status, duration of asthma, age, frequency of admission and steroid dosage. Parents' profiles were their age, number of asthmatic children and the level of asthma knowledge.
Result: Fifty-six (83.5%) parents identified more than one trigger factor for their children's exacerbation. The commonest frequency was two trigger factors (31.3%). Upper respiratory tract infection (77.6%) was the commonest trigger factor. There was no association between the number of trigger factors with the severity of asthma and level of parental asthma knowledge. The number of trigger factors significantly correlated with asthma duration (r = 0.33, p = 0.006). The asthma severity was associated significantly with weather (p = 0.042) but not with other trigger factors.
Conclusion: The majority of parents recognised more than one trigger factors to cause asthma exacerbation.
An eight-year-old Chinese girl presented with a slowly progressive generalized muscle weakness and wasting, complicated by respiratory failure. She had many hospital admissions requiring ventilator support. Eventually tracheostomy tube was inserted. Initial investigations failed to elicit a diagnosis but a muscle biopsy and histological study confirmed the diagnosis of juvenile acid maltase deficiency.
Even though vaccines are effective for prevention of diseases, compliance with recommended schedules of immunisation in early childhood is not always complete. Identification of potential risk factors associated with under-immunisation may help increase vaccination uptake. The objectives of this study were to determine the prevalence and risk factors of under-immunisation in hospitalised preschool children in Universiti Sains Malaysia Hospital (HUSM). Children aged 1 month to 2 years admitted between December 1998 and December 2000 in medical paediatric wards at HUSM were studied. Parents/guardians were interviewed during admission regarding immunisation status and potential risk factors. Children who did not have home-based cards during admission were excluded. Among 401 children, 37.9% had complete immunisation, 43.4% had delayed immunisation and 34.9% had incomplete immunisation. The uptake of BCG, DPT/OPV 1, DPT/OPV2, Hepatitis B3, DPT/OPV3, and measles were 98.5%, 90.7%, 83.2%, 81.4%,79.2% and 72.9% respectively. Vaccines scheduled to be given at later ages were significantly less likely to be taken (p=0.002) compared with vaccines scheduled to be given at an earlier age. Significant independent risk factors for under-immunisation were elderly mothers (3 40 years old) (OR 3.0, 95% CI: 1.2-8.0), low birth weight £2.0 kg (OR 6.2; 95% CI: 1.8-21.4) and presence of chronic illness in the child (OR 4.5; 95% CI: 2.5-7.9). In children whose father's education was low (primary school or no education), low maternal education was significantly associated with lower rate of under-immunisation (p = 0.005, OR 0.11 95%CI 0.02-0.51).
In conclusion this study showed that a large proportion of hospitalised preschool children were under-immunised, and vaccines scheduled to be given at a later age had lower uptakes. The situation can be rectified if every opportunity is taken to immunise all children, who have lapsed in their immunisation schedule.
Heralded by the revelation of the double helical structure of the DNA molecule in 1953, the 21st century is aptly designated the biotechnology century. The 20th century of physics, which saw the transformation of silicon into computing magic, was embraced with enthusiasm by virtually every household. However, unlike her predecessor, the same cannot be said about the advancements in biomedicine.
Endobronchial tuberculosis is an uncommon manifestation involving the tracheobronchial tree. The clinical presentation is typically non-specific. We report this unusual complication of pulmonary tuberculosis initially diagnosed as foreign body in a 16-month-old child.
We report a rare case of a newborn baby girl who was delivered at 36 weeks of gestation with birth weight of 2680 grams and has ankyloblepharon filiforme adnatum with cleft lip and palate diagnosed at birth. The baby was managed with simple procedures of eyelid bands separation. We also reviewed the embryology of eyelid and its relation with ankyloblepharon formation and discussed the various syndromes and abnormality which may be associated with this eyelid abnormality. The four types of ankyloblepharon which was described by Rosenman and colleague is presented.
A case control study was conducted to identify the risk factors for acute lower respiratory tract infections (ALRI) in hospitalised children in Kelantan. One hun-dred and twenty children aged one month to five years hospitalised for ALRI were matched by age with 120 children as controls. Data on demography and expo-sure to putative risk factors were collected by interview-ing parents or caretakers. Anthropometric measure-ments were also carried out to assess the nutritional sta-tus of the children. For each risk factor studied, the odds ratios for exposure and disease were calculated by using univariate analysis followed by multiple logistic regression analysis to determine those factors which remained significant.
The presence of sibling(s) who coughed at home (OR 12.1, 95% CI 5.2-28.1), crowding in bedroom (OR 4.4, 95% CI 2.1-9.0), weight-for-age < 3rd percentile (OR 9.0, 95% CI 3.1-25.8), lack of breast feeding (OR 9.4, 95% CI 2.3-38.4) and incomplete immunisation (OR 4.5, 95% CI 1.7-12.1) were significant indepen-dent risk factors for ALRI. Other factors like poverty, maternal education level, male sex, low birth weight, history of atopy, family history of asthma and indoor air pollution were not associated with an increased risk of ALRI.
This study showed that poor nutritional status, inap-propriate child care practices and poor living conditions, particularly those related to crowding, predispose to ALRI in Kelantanese children necessitating hospital admission. A change in these factors may reduce the morbidity and mortality of childhood ALRI in Kelantan.
A 46 day old female Chinese infant was referred for fail-ure to thrive, jaundice, hepatomegaly and bilateral cataracts. She had vomiting,blood stained stools and severe unconjugated hyperbilirubinaemia soon after birth. The jaundice persisted. At one month of age, pale stools, firm hepatomegaly and bilateral cataracts were noted. Radionuclide hepatobiliary scintigraphy per-formed at another hospital excluded biliary atresia. Investigations showed cholestasis but a negative sero-logical screening for congenital infections. A presump-tive diagnosis of galactosaemia was made and the infant was started on lactose free formula. A deficient red blood cell galactose- 1 -phosphate uridyltransferase (GALT) activity was demonstrated later. Review eight months after the initial diagnosis showed a thriving infant with no jaundice, but persisting cataracts and firm enlarged liver. A high index of clinical suspicion, labo-ratory confirmation of a deficient GALT activity and prompt withdrawal of lactose from diet are necessary to avoid any delay in diagnosis and management of this condition.