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.

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.

The human genome contains many submicroscopic copy number variations which includes deletions, duplications and insertions. Although conventional karyotyping remains an important diagnostic tool in evaluating a dysmorphic patient with mental retardation, molecular diagnostic technology such as array comparative genomic hybridization (aCGH) has proven to be sensitive and reliable in detecting these submicroscopic anomalies. A 3 month-old infant with dysmorphic facies, microcephaly and global developmental delay was referred for genetic evaluation. Preliminary karyotyping which was confounded by the quality of metaphase spread was normal; however, aCGH detected a 30.6Mb deletion from 5p15.33-p13.3. This case illustrates the usefulness of aCGH as an adjunctive investigative tool for detecting chromosomal imbalances.