Displaying publications 61 - 80 of 117 in total

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  1. Ghazali, F., Jamal, R., Zakaria, S.Z., Ismail, Z.H., Malik, Y.
    MyJurnal
    The two vital aspects of treatment for patients with tha-lassaemia are regular blood transfusions and iron chela-tion therapy. Unfortunately, the use of blood transfu-sions exposes these patients to the risks of acquiring transfusion related viral infections such as hepatitis C. Patients who acquire the hepatitis C virus (HCV) may develop chronic hepatitis and later on hepatocellular carcinoma. Hence, patients with thalassaemia should be regularly screened for the presence of HCV. We report here the results of a cross-sectional study conducted in a typical day-care centre for thalassaemics at the Hospital Universiti Kebangsaan Malaysia, involving 85 multiply transfused patients. We found that 19 patients (22.4%) were seropositive for HCV and two of them had positive HCV-RNA. Those who had started receiv-ing their transfusions before 1995, i.e. the year routine screening for HCV amongst blood donors were com-menced, and those who received transfusions 2-4 week-ly had a significantly higher risk of acquiring HCV infection.
  2. Lim, W.L., Lim, C.T., Chye, J.K., Ho, M.M.
    MyJurnal
    The objective of this study was to examine the effect of surfactant replacement therapy on hospital resource uti-lization in a well defined cohort of preterm infants with respiratory distress syndrome (RDS). Thirty preterm infants 800g with RDS requiring mechanical ventila-tion with Fi02 of 0.4 given modified bovine surfactant (Survanta) were compared with an equal number of his-torical controls. The total cost of neonatal care was cal-culated in a detailed survey covering all aspects of resource use. Surfactant-treated infants had an improved survival rate (90.0% vs 66.7%, p=0.03) and a trend towards shorter ventilator days (11.8 vs 19.0 days, p=0.17). There were no significant differences in the number of laboratory and radiological investiga-tions, use of disposable items, equipment, medications and other therapies. The total hospital cost per livebirth for surfactant-treated and control infants were R/V120,281 and R1V121,785 respectively. Personnel salaries represented the largest sector of resource uti-lization. When analysed by birthweight categories, the cost per livebirth for surfactant-treated and control infants in the 800-999g category were RM37,315 and RM14,760 respectively. As for the surfactant-treated and control infants in the 1000-1499g category, the cost per livebirth were RM21,426 and RM32,327 respectively. We concluded that surfactant replacement therapy did not increase overall hospital resource uti-lization and may decrease the cost for infants weighing 1000g.
  3. Mohd Yusoff, N., Choo, K.E., Ghazali, S., Ibrahim, I., Mohd Hussin, Z.A., Mohd Yunus, et al.
    MyJurnal
    Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked red blood cell enzymopathy common in malaria endemic areas. Individuals affected by this disease show a wide variety of clinical signs including neonatal jaundice. In this preliminary report we describe the heterogeneity of G6PD deficient gene in neonatal jaundice in the Malay population in Kelantan. Thirteen G6PD deficient Malay neonates with hyperbilirubinemia were subjected to mutation analysis of the G6PD gene for known candidate mutations. Molecular defects were identified in the 13 patients studied. Though all of these were mis-sense mutations, identified nucleotide changes were heterogeneous. Six patients were found to have a C to T nucleotide change at nucleotide 563 of the G6PD gene (C563T), corresponding to G6PD Mediterranean; three cases had a single nucleotide change at T383C (G6PD Vanua Lava), two cases had G487A (G6PD Mahidol) and two cases had G1376T (G6PD Canton). These findings suggest that there are heterogeneous mutations of the G6PD gene associated with neonatal jaundice in the Malay population in Kelantan.
  4. Singh, H.
    MyJurnal
    Clubfoot is associated with leg length inequality, with the affected leg being shorter as a consequence of the hypoplasia. Early treatment of a minimally hypoplastic clubfoot usually allows catchup growth and both legs equalise in length after a few years. Late treatment however is associated with a shortening of the affected foot. A case is described where late treatment of a severe clubfoot is assocated with lengthening of the affected leg.
  5. Singh, H.
    MyJurnal
    It is very rare indeed in the practice of children's orthopaedics today in Malaysia that the natural history of long bone osteomyelitis is seen. A case is presented where a shoulder abscess in a 3-year-old child developed into septic arthritis of the shoulder and subsequently chronic osteomyelitis of the adjacent humerus. The parents refused active surgical debridement and sequestrectomies. Three years later the child had regenerated a new humerus over the sequestra. Radiographs are presented illustrating the entire natural history of osteomyelitis with the regeneration of a new humerus.
  6. Menon, B.S., Mohamed, W.M., Majid, N.A., Ariff, A.R.
    MyJurnal
    We report a case of chemotherapy induced acute pan-creatitis in a child with acute lymphoblastic leukaemia. L-asparaginase is the most likely incriminating drug.
  7. Mohamed, M., Ariffin, H., Arasu, A., Tuck Soon, S.H., Abdullah, W.A., Lin, H.P.
    MyJurnal
    Fusarium species is an emerging genus of fungal pathogens which until recently were rare causes of human disease apart from localized infection of the skin and nails. Two cases of fungaemia due to Fusarium sp. in children are described. The first child, an 8-year old girl with acute myeloid leukaemia developed character-sitic pyoderma gangrenosum-like skin lesions before succumbing to disseminated Fusarium infection and acute respiratory distress syndrome. The second child, a 5-month old boy, developed pneumonia associated with a transient erythematous skin rash while on chemother-apy for congenital leukaemia. Both patients had Fusarium isolated from blood. The second child improved after six weeks of treatment with ampho-tericin B and granulocyte-macrophage colony stimulat-ing factor but ultimately she died of the disease follow-ing discharge. Fusarium spp should be recognised as an opportunistic pathogen in immunocompromised patients. Current literature suggests that liposomal amphotericin B in conjunction with leukocyte growth factors are the treatment of choice in this potentially fatal infection.
  8. Boey, Christopher C.M.
    MyJurnal
    Helicobacter pylori (HP) was first described in 1983 by Warren and Marshall.' It is a spiral-shaped bacterium measuring 2-4p,m x 0.5-1.0μm. Since it was discovered, the organism has rarely been isolated from sites other than the stomach.2 Available evidence, therefore, points to the human stomach as the normal habitat of this bacterium. The infection is contracted primarily in childhood.' It has been shown that colonisation by HP is rare under the age of five years, but thereafter, it becomes gradually more frequent, and by sixty years of age more than 50% of individuals may be affected
  9. Norlijah Othman, Nor Khamisah, A., Kamarul Azhar, Sinniah, Mangalam, Lekhraj, Rampal
    MyJurnal
    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.
  10. Rohana, J., Latinah, M., Thambidorai, C.R., Kaur, B.
    MyJurnal
    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.
  11. Musa, M.N.
    MyJurnal
    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.
  12. Norhasimah, M.M., Ahmad Tarmizi, A.B., Azman, B.A., Zilfalil, B.A., Ankathil, R.
    MyJurnal
    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.
  13. Norlijah, O., Abu, M.N., Mohd Nor, A., Yip, C.W.
    MyJurnal
    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.
  14. Thong, M.K., Choy, Y.S., Rawi, R.M.
    MyJurnal
    Inborn errors of metabolism (IEM) are a group of disorders that causes abnormal function of biochemical pathways. Archibald Garrod des-cribed the first inborn error of metabolism in 1893. He described alkaptonuria in a patient whose urine turned black on standing and the development of arthritis in adult life.' Subse-quently, Garrod encapsulated the idea of IEM in 1908 with the concept of 'chemical indivi-duality'. Beadle and Tatum proposed the concept of one gene - one enzyme in 1945.2 Phenyl-ketonuria (PKU) was described in 1934 and amongst the first to be recognised as a cause of mental handicap with a biochemical basis.' Effective treatment for PKU with low pheny-lalanine diet was introduced in 1955. Molecular characterisation of genetic defects localised to alleles in various chromosomes were performed in the last two decades
  15. Chan, P.W.K., Cheong, B., Nadarajan, K., Lai, B.H., Cham, W.T., Khoo, K.K., et al.
    MyJurnal
    The objective of the study was to determine the prevalence of hypertension in healthy primary school children. A cross-sectional survey by manual blood pressure measurement of healthy children aged 6-12 years attending a national type school was carried out. Children with previous renal, cardiac and endocrine disorders were excluded. Korotkoff 1 represented the systolic blood pressure (SBP) and Korotkoff 5 was taken as the diastolic blood pressure (DBP). Hypertension was defined as the SBP or DBP above the 95th centile according to age group and sex regardless of ethnicity. A total of 1756 children were studied with 895 boy and 861 girls. The results showed that 109 children (6.2%) were found to have hypertension and the incidence was similar in boys and girls (6.4% vs 6.0%, p = 0.69). Fifty-three (48.6%) children had an elevated SBP and the DBP was elevated in 43 (39.4%) children. Only 12 (12.0%) children had both elevated SBP and DBP. Children found to have hypertension were more likely to be Chinese (p = 0.009) and obese (p = 0.04). In conclusion the overall prevalence of hypertension in children aged 6-12 years based on a single blood pressure measurement was 6.2%. Hypertension was more likely to be found in Chinese children and those who were obese.
  16. Adele, Tan Guat Kean
    MyJurnal
    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
  17. Fatemeh, H., Watihayati, M.S., Marini, M., NurShafawati, A.R., Atif, A.B., Zabidi-Hussin, Z.A.M.H., et al.
    MyJurnal
    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.
  18. Hung, Liang Choo
    MyJurnal
    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.
  19. Irfan Mohamad, Abdul Halim Shibgatullah, Rosdan Salim
    MyJurnal
    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.
  20. Alam Sher Malik, Rukhsana Hussain Malik
    MyJurnal
    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.
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