Juvenile myelomonocytic leukaemia (JMML), previously known as juvenile chronic myeloid leukaemia (JCML) is a rare, myelodysplastic – myeloproliferative disease typically presenting in early childhood. This disorder is diffi cult to distinguish from other myeloproliferative syndrome such as chronic myeloid leukaemia (CML) because of the similarities in their clinical and bone marrow fi ndings. However, because of its unique biological characteristics such as absolute monocytosis with dysplasia, absence of Philadelphia chromosome or BCR-ABL fusion protein, hypergammaglobulinaemia and raised fetal haemoglobin level, this disorder does not satisfy the criteria for inclusion in the CML or chronic myelomonocytic leukaemia (CMML) group, as seen in adult patients. We describe a series of three patients with JMML, who had almost similar clinical and laboratory fi ndings, and discuss the diffi culty in the classifi cation and treatment of the disease.

Residual disease in patients with acute leukaemia indicates unfavorable prognosis. The evaluation of remission using flow cytometry allows a better estimation of minimal residual disease (MRD) after induction chemotherapy in childhood acute lymphoblastic leukaemia (ALL) cases. Patients in morphological marrow remission with presence of blast cells of less than 5%, may still have up to 1010 leukaemic cells. However with flow cytometric analysis, lower levels of the residual leukaemic cells (1 in 104 cells) can be detected and it can be used as a tool to predict relapse. This study compared the presenting clinical and haematological features of children with ALL and their residual disease status determined by flow cytometry. Analysis of their MRD status following remission-induction chemotherapy were done at day-28, week-12 and week-20. The cases were also followed up to five years, to determine their survival status. Their residual disease status by flow cytometric immunophenotyping was also compared with their bone marrow findings morphologically. Thirty-eight cases of precursor B-ALL in pediatric patients from UKM Medical Centre (UKMMC) were analyzed. There was no significant correlation between demographic, clinical and haematological features with MRD status at day-28. However, there was a significant correlation between MRD status by flow cytometry and by morphological marrow examination at week-12. Three cases showed persistent MRD findings until week-20 where two of the cases relapsed and died subsequently. Twenty four patients were still alive after five years of follow up.

Lately, increasing workplace violence is a problem that requires serious attention by the management of the healthcare sector. This study was carried out to examine workplace violence experienced by nurses in the Universiti Kebangsaan Malaysia Medical Centre. It aimed to identify the category of nursing staff who formed the highest number of victims, the perpetrators and the common types of workplace violence. This was a descriptive cross-sectional study carried out over a three month period on 55 nurses who had participated voluntarily in this study. The demographic data and data on the violent incident were collected by using a 21-item questionnaire. The reliability and validity with Cronbach’s alpha was 0.872. Information on the most common types of violence, victims and the perpetrators was also collected. The study revealed that workplace violence among the nursing staff was 3.7% with an average of 1.2% being abused per month and one nurse being abused every other day. The study also revealed that the staff nurses were the most common victims and the perpetrators were mainly the patients (40.6%) and the patients’ relatives (37.5%). The most common forms of violence were verbal abuse (31.9%) and verbal threat (23.7%). Physical violence was experienced by 44.4% of the victims. These results suggest that workplace violence is a serious problem which should be addressed in order to create a safe environment for the nurses.

Ventilated patients who require intensive care unit (ICU) treatment may encounter unpleasant experiences. These experiences may include factual incidents and delusional memories of ICU such as dreams, hallucinations and frightening experiences. A cross sectional study using “Intensive Care Experience Questionnaire” consisted of four domains: awareness of surrounding, frightening experiences, recall of experience and satisfaction with nursing care. Forty five participants who fulfilled the inclusion criteria were recruited in this study. This study was conducted in ICU of HUKM from January to March 2006. Results showed that 20 respondents (44%) were aware of their surrounding and 31 respondents (69%) reported frightening experiences. Majority of respondents (43 respondents, 96%) reported satisfaction with the delivery of nursing care. There was positive correlation between awareness of surrounding and their abilities to recall their experiences (p<0.05). Patients’ awareness of surrounding achieved the strongest statistical significance as a contribution to the prediction of their abilities to recall their experiences with beta coefficient value of 0.353 and p<0.05. The finding of this study permits nurses to see the problems of mechanically ventilated patients through reports of unpleasant recollections by the patients themselves. Patients reported frustrations in their attempts to make their needs known. Although they are on sedation, they are aware of all unpleasant events occurring in the ICU

Parenting stress is the stress level experienced within the role of a parent (HoekstraWeebers et al. 1998). The source of stressors is variable and dependent on the phase of disease and chemotherapy (Sawyer et al. 2000). Failure to cope with these stressors may in turn affect the child’s emotional and social adjustment towards the diagnosis of cancer in addition to poor medical treatment adherence behaviour(Sawyer et al. 1993). The objectives of this study are to determine the level of parenting stress, the risk factors contributing to high parenting stress, and the coping mechanisms used to handle the stress. This single centred, cross-sectional study was done amongst 117 parents at the Paediatric Haematology and Oncology Unit, Universiti Kebangsaan Malaysia Medical Centre (UKMMC) over two years duration. Self-administered questionnaires comprising the Parenting Stress Index/Short Form (PSI/SF) and Coping Inventory for Stressful Situation (CISS) were distributed to parents of children who were 12 years old and below. The mean total parenting stress score amongst parents of children diagnosed with acute leukaemia was 91.5±21.1(95%CI). A total of 27.3% of parents experienced a high total parenting stress score(defined as total PSI score ≥ 75th centile, ie ≥ 103). Task-oriented coping mechanism was used by the majority of parents. Emotion-oriented coping mechanism was the only identifiable risk factor for high parenting stress score following multiple logistic regression analysis. A parent who used emotion-oriented coping mechanism was 7.1 times (95% Confidence Interval 1.2 to 41.4) more likely to have a high parenting stress score compared to a parent who used other coping mechanisms. By identifying these at-risk parents, appropriate counselling and psychological support may be offered early to alleviate the stress as well as assist in the coping and adjustment mechanisms of these parents.

Child ; Folic Acid ; administration & dosage ; therapeutic use ; Folic Acid Antagonists ; adverse effects ; Hematinics ; administration & dosage ; therapeutic use ; Humans ; Hypesthesia ; chemically induced ; drug therapy ; Injections, Spinal ; Leukoencephalopathies ; chemically induced ; Male ; Methotrexate ; adverse effects ; Quadriplegia ; chemically induced ; drug therapy ; Time Factors ; Vitamin B Complex ; administration & dosage ; therapeutic use

Molecular pathogenesis of chronic myeloid leukemia (CML) is well established and molecular monitoring for patients with CML has become an important practice in the management of patients on imatinib therapy. In the present study, we report the use of RQ-PCR method for detection of BCR-ABL fusion gene for our CML cases. We performed a two-step RQ-PCR on bone marrow aspirates or peripheral blood of 37 CML patients. Quantitative expression of BCR-ABL fusion gene was carried out relative to the expression of a housekeeping gene as endogenous control to compensate for uneven cell numbers, RNA quality, or variations in reverse transcription effi ciencies. Twenty-four of these patients were pre-treated with hydroxyurea or alpha interferon prior to the imatinib therapy. Their BCR-ABL fusion gene levels were monitored for 18 months. All samples processed were evaluable. The PCR amplifi cation effi ciency of the ABL gene is 90.5% (0.2158) and the BCR-ABL gene, 93.4% (0.1573).

Objective: This study aimed to evaluate the prevalence of sensorineural hearing loss (SNHL) in β-thalassaemia patients treated with Desferrioxamine (DFO) and determine the correlation of SNHL with average daily DFO dosage, serum ferritin level and Therapeutic index (T.I). Methods: This is a cross sectional descriptive study carried out for a period of 14 months and 54 patients were recruited. The recruited patients are transfusion dependant β- thalassaemia patient aged 3 years and above treated with DFO. An interview, clinical examination and hearing assessment, which included tympanogram, and Pure Tone Audiometry (PTA) or behaviour alaudiometry were performed. The data on age started on DFO, average daily DFO, duration of DFO intake, serum ferritin past 1 year and Therapeutic Index (T.I) were obtained from patients’ case notes. Results: The prevalence of SNHL was 57.4% and majority has mild hearing loss (93.6%). Fourteen patients (25.9%) have bilateral ear involvement and as many as 17 patients (31.5%) have SNHL in either ear. A total of 23 patients (42.6%) have normal hearing level. Although the prevalence of SNHL was 57.4%, only a small percentage of the patient noticed and complained of hearing loss (11.1%). There is no association between age started on DFO, average daily DFO and duration of DFO intake with normal hearing group and those patients with SNHL. Positive correlation was seen between average daily DFO with 2000 and 4000Hz on PTA in the left ear and between serum ferritin level past 1 year with 4000 and 8000Hz in the right ear and 8000Hz in the left ear. No significant correlation was seen between T.I on PTA. Conclusion: The prevalence of SNHL from hearing assessment is high in β-thalassaemia patients in this study. However, it is manifested clinically in a smaller percentage. We suggest a baseline hearing assessment should be carried on all β-thalassaemia patients prior to DFO chelation therapy.

Antibody screening is important for the antenatal screening and pre-transfusion tests. This study aimed to compare the MUT/Mur kodecytesAbtectcell III (CSL Abtectcell III) red cell antibody screening kit with DiaMed ID-Dia Cell I-II-III Asia that was then used in our laboratory. In this study, 125 samples were randomly chosen, with 67 samples of known antibody specificities and 58 samples identified as negative for antibody, as the negative control. Concordant negative results were obtained in 57 out of 58 antibody negative samples. Concordant antibody positive results with both reagents were seen in 49 out of 67 samples. There were 18 discrepant results of antibody screening with CSL Abtetcell III (16/18 for vMNS antibodies). The sensitivity and specificity for CSL Abtectcell III were 73.0% and 98.3% respectively. In conclusion, the CSL Abtectcell III reagent would be an acceptable alternative for screening of red cell alloantibodies. It was able to detect all the clinically significant alloantibodies.

Information technology use in healthcare education has become a popular medium of instruction. One of the medium of instruction is video assisted learning (VAL). The use of VAL as an instructional method in the teaching and learning of emergency skills is not new. However, there are lack of studies on the perception of using this method in learning emergency skills. This qualitative study involved four focused discussion groups following a VAL instruction on emergency skills. A total of 20 paramedics were divided into four groups. They were involved in a focussed discussion after a VAL instruction session. Findings reveal that the paramedics perceived three major themes which were categorized as : i) advantages of video as teaching tool, ii) barrier in using video as a teaching tool; and iii) suggestions on using video as teaching tool. The findings indicate that the paramedics perceived VAL as a potential tool for learning emergency skills. However, they suggested the language of instruction should be in their mother tongue for better understanding. This implies that using English language has disadvantage in technology enhanced learning for better understanding.
Computer-Assisted Instruction