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Fulltext Survival of Children with Acute Leukaemia: A Single Centre Experience

Ariffin Nasir, Nor Fadhilah Zahari, Fahisham Taib, Norsarwany Mohamad

Malaysian Journal of Paediatrics and Child Health, 2020;26(1):4-13.
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Introduction: Acute leukaemia in children accounts for 25-30% of malignant diagnosis. Survival from acute leukaemia continue to improve. Treatment outcome depends on factors like gender, age at diagnosis, parental education, the initial total white cell count (TWC), cerebrospinal fluids (CSF) infiltration, immunophenotype and treatment response. Objectives: The objectives were to evaluate the survival of children with acute leukaemia who received chemotherapy and identify relevant factors. Methodology: The study was a retrospective record review at the Paediatric Oncology Unit, Hospital Universiti Sains Malaysia (Hospital USM). The data collected depending on pre-set research proforma from the year 1990 to 2010. Survival analysis of each type of leukaemia was completed using multiple Cox regression model. Results: A total of 334 cases were identified, only 283 patients received treatment at Hospital USM. There were 224 patients with acute lymphoblastic leukaemia (ALL) and 59 with acute myeloid leukaemia (AML). Overall survival (OS) rate at 3 months for ALL and AML were 89.3% and 72.9% respectively. The event-free survival (EFS) rate for ALL at 1, 3, and 5 years were 69.6%, 54.1% and 47.8% respectively. For AML, the EFS rate at 1, 3, and 5 years were 52.0%, 42.4% and 38.1% respectively. Multiple Cox regression model showed children’s age at diagnosis and early response to steroid therapy were the most significant prognostic factors for ALL survival, whereas the spleen size and treatment protocol were the most significant prognostic factors for AML. Conclusion: Survival rate in this study was comparable to developing countries. ALL had better outcome compared to AML.
Fulltext Can I give food or drink to my terminally ill child?

Taufiq Hidayat, Zahoor Iqbal, Ariffin Nasir, Norsarwany Mohamad, Fahisham Taib

Education in Medicine Journal, 2015;7(3):73-78.
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Food is considered as a social responsibility of caregivers to their children. It has cultural connotation for all races notwithstanding their background and religious belief; that social responsibility should not be separated even in terminally ill patients. We recorded a case scenario of a terminally ill child who faced difficult end of life with inability to take oral fluids or food due to mechanical obstruction of duodenum by the pelvic rhabdomyosarcoma. From cultural context, the physical act of giving food and fluids to a sick person is considered “a display of one’s affection”. It is understandable that, once the dying phase has reached, and the body starts to shut down, administering fluids may not be useful despite it is deemed necessary from cultural point of view. This case illustrates an ethical dilemma in managing a child with end stage metastatic disease with multiple systemic complications, compounded with futility of medical care and complex social circumstances. It is quite challenging for physicians and relatives to provide good end of life care to patients in palliative care setting. Ensuring good quality of care, quality of life and quality of death are paramount to avoid suffering and distress among the patients and family members.
Fulltext Plasma lead levels among paediatric thalassaemic patients in Hospital Universiti Sains Malaysia, Kelantan, Malaysia

Iyen, Mohd I., Ab Wahab, Mohd S., Norsarwany Mohamad, Mariani Mohamad, Erejuwa, Omotayo O.

Malaysian Journal of Paediatrics and Child Health, 2016;22(1):17-25.
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Objective: Lead toxicity constitutes a major environmental risk to health in both animals and humans of all ages. It is more severe in young children than adults. Blood transfusion is an important source of lead exposure and may predispose premature infants to lead toxicity. Thalassaemia is common in Malaysia and majority of patients require frequent blood transfusion. The objective of this study was to determine whether regular blood transfusion contributed to high blood lead levels in paediatric thalassaemic patients.

Method: This was a cross sectional study conducted at the Paediatric Thalassaemia Day Care Unit, General Paediatric Ward and Paediatric Clinic in Hospital Universiti Sains Malaysia (HUSM). A total of 90 patients were included, 45 were thalassaemic transfusion dependant patients and the other 45 were control, who were of the same age and sex with patients group and had never been transfused. The blood samples were taken preand post-transfusion for thalassaemic and control groups. Blood lead levels were analyzed using standard Atomic Absorption Spectrometer (AAS) analysis.

Results: The overall mean plasma lead levels (2.13 + 1.72µg/dL) were lower than those of standard CDC recommendations. The independent t-test showed that plasma lead levels in thalassaemic group were significantly (p < 0.05) lower than the levels in controls. However, the ANCOVA analysis revealed the plasma lead levels were not significantly (p > 0.05) different between the two groups. Thus, suggesting that the reduced plasma lead level in thalassaemic group was due to the administration of iron chelators. Increased frequency of blood transfusion also did not significantly (p > 0.05) increase plasma ferritin or lead levels in thalassaemic patients.

Conclusion: This study shows that transfusion dependent thalassaemic infants have comparable plasma lead levels to those of age- and sex-matched controls, after taking into consideration the administration of iron chelators.

Study site: Paediatric Thalassaemia Day Care Unit, General Paediatric Ward and Paediatric Clinic in Hospital Universiti Sains Malaysia (HUSM)
Fulltext The relationship between socio-demographic and illness-related variables with the quality of life among Malaysian adolescent with thalassaemia: a multi-centre study

Azizah Othman, Qarem Mohamed Mustafa, Ariffin Nasir, Norsarwany Mohamad, Nurul Shafira Adi, Nurul Ilyana Hashim, et al.

Malaysian Journal of Paediatrics and Child Health, 2018;24(1):15-26.
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Thalassaemia is a life-long illness that exists globally. The quality of life of adolescents with thalassaemia could differ based on the health policies of a specific region, existing levelof socio-economic development and the illness related variables. This study examines the relationship between socio-demographic and disease-related variables with the quality of life among adolescents with thalassaemia involving multiple treatment centers spread throughout various locations in Malaysia. Participants included 218 adolescents (male=108; female 112) with mean age of 13.86 (SD=2.40). They completed the questionnaire consisting of demographic information, illness-related variables, and Pediatric Quality of Life Inventory 4.0 (PedsQL). The participants in this study was found to have higher total summary score (Mean = 69.64, SD = 14.03), psychosocial health (Mean = 70.23, SD = 14.91), emotional (Mean = 72.12, SD = 20.66), social (Mean = 79.82, SD = 17.37), and school (Mean = 58.69, SD = 16.77) functioning but with lower physical health (Mean = 68.50, SD = 17.22) as compared to previous study that was done in Kuala Lumpur. Findings also shows a significant positive correlation between level of education and frequency of hospitalization (r = .156, p < 0.05), frequency of transfusion (r = .152, p < 0.05), and physical health (r = .186, p < 0.01). An increase in the frequency of transfusion was found to significantly increase social functioning (r = .137, p < 0.05). Other significant correlations are discussed in addition to the quality of life experienced by patients with thalassaemia in different region of theworld.
Fulltext Combination of Pyridoxine and Thiamine Treatment in Bilateral Induced Ptosis in a Child: Case Report and Review of Literature

Chai KS, Norsarwany M, Shatriah I

Cureus, 2017 Aug 16;9(8):e1573.
PMID: 29057185 DOI: 10.7759/cureus.1573

Ptosis is a rare side effect of vincristine chemotherapy in patients treated for cancer. We report a case of a child with common B-cell acute lymphoblastic leukemia who developed bilateral moderate ptosis following the chemotherapy protocol of the United Kingdom Acute Lymphoblastic Leukemia (ALL) regimen A. The patient showed dramatic clinical improvement after a combination of oral pyridoxine and thiamine treatment. We provide a literature review of this uncommon presentation.
Prognostic factors and survival rate of osteosarcoma: A single-institution study

Faisham WI, Mat Saad AZ, Alsaigh LN, Nor Azman MZ, Kamarul Imran M, Biswal BM, et al.

Asia Pac J Clin Oncol, 2017 Apr;13(2):e104-e110.
PMID: 25870979 DOI: 10.1111/ajco.12346

AIM: Osteosarcoma is a highly malignant primary bone tumor. The study aim to evaluate the prognostic factors influencing the survival rate in our center.
METHODS: This was a retrospective cohort study of all patients treated between January 2005 and December 2010.
RESULTS: We included 163 patients with an age range of 6-59 years (median = 19). The median follow-up was 47 months (range 36-84). The overall survival in patients who completed chemotherapy and surgery (n = 117) was 72% at 2 years and 44% at 5 years. Histologically, 99 (85%) had osteoblastic, 6 (5%) had chondroblastic and 3 (2.5%) had telangiectatic osteosarcoma. Limb salvage surgery was performed in 80 (49%) and 41 (25%) underwent amputation. However, 46 patients (28%) underwent no surgical intervention and incomplete chemotherapy. In total, 38/79 patients had a good chemotherapy response. There was a significantly better survival rate for limb salvage versus amputation. Independent prognostic factors for survival are compliance to treatment and presence of lung metastasis.
CONCLUSION: The overall survival of osteosarcoma patients was influenced by the presence of pulmonary metastases and compliance to treatment. Histological subtype, different chemotherapy regimens and histological necrosis after chemotherapy did not significantly influence survival. The patients who did not complete treatment had significantly poorer survival.