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

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.

Haemoglobin (Hb) Lepore is a variant Hb consisting of two α-globin and two δβ-globin chains. In a heterozygote, it is associated with clinical findings of thalassaemia minor, but interactions with other haemoglobinopathies can lead to various clinical phenotypes and pose diagnostic challenges. We reported a pair of siblings from a Malay family, who presented with pallor and hepatosplenomegaly at the ages of 21 months and 14 months old. The red cell indices and peripheral blood smears of both patients showed features of thalassaemia intermedia. Other laboratory investigations of the patients showed conflicting results. However, laboratory investigation results of the parents had led to a presumptive diagnosis of compound heterozygote Hb Lepore/β-thalassaemia and co-inheritance α+-thalassaemia (-α3.7). Hb Lepore has rarely been detected in Southeast Asian countries, particularly in Malaysia. These two cases highlight the importance of family studies for accurate diagnosis, hence appropriate clinical management and genetic counseling.

Haemoglobin (Hb) Lepore is a variant Hb consisting of two α-globin and two δβ-globin chains. In a heterozygote, it is associated with clinical findings of thalassaemia minor, but interactions with other haemoglobinopathies can lead to various clinical phenotypes and pose diagnostic challenges. We reported a pair of siblings from a Malay family, who presented with pallor and hepatosplenomegaly at the ages of 21 months and 14 months old. The red cell indices and peripheral blood smears of both patients showed features of thalassaemia intermedia. Other laboratory investigations of the patients showed conflicting results. However, laboratory investigation results of the parents had led to a presumptive diagnosis of compound heterozygote Hb Lepore/β-thalassaemia and co-inheritance α+-thalassaemia (-α3.7). Hb Lepore has rarely been detected in Southeast Asian countries, particularly in Malaysia. These two cases highlight the importance of family studies for accurate diagnosis, hence appropriate clinical management and genetic counseling.

Malnutrition is a common occurrence during cancer treatment among pediatric cancer patients and without proper care, it can bring negative implications. The purpose of this study was to develop a printed educational material titled “Penjagaan Pemakanan untuk Penjaga Kanser Kanak-Kanak” (Nutrition Care for Children with Cancer) to help the caregivers to manage their child in terms of nutrition. The objective of this study was to evaluate caregiver’s knowledge, attitude and practice (KAP) before and after receiving the printed educational material and to evaluate the acceptance of the printed educational material among caregiver. This quantitative study (Quasi-Experiment) was conducted among pediatric oncology caregivers in Institute of Pediatrics Hospital Kuala Lumpur (IPHKL)(n=34) and Universiti Kebangsaan Malaysia Medical Center (UKMMC)(n=34). IPHKL served as the experimental group where the caregivers received the developed booklet meanwhile UKMMC served as the control group where the caregivers received a pamphlet published by Institut Kanser Negara titled “Terapi Diet Pesakit Kanser – Kesan Sampingan Rawatan dan Cara Mengatasinya” (Nutrition Care for Cancer – How to Overcome the Side Effects of the Treatment). Results from this study showed that there was a significant increase (p<0.05) for KAP of the caregivers in both groups with a higher increase was seen in the experimental group. The score for KAP in the experimental group was (70 ± 8%) and after receiving the printed educational material, the score significantly increased to (92 ± 10%) which was higher compared to control group where their score before was (70 ± 11%) and increased to (77 ± 10%). For the evaluation of acceptance, there was a significant difference (p<0.05) for the mean score between experimental group (48.85 ± 4.931) and control (42.29 ± 6.834) with t= 4.538. In conclusion, the printed educational material developed from this study was well accepted and has the potential to effectively increase caregivers’ nutritional knowledge of managing the nutritional aspect of pediatric cancer patients during treatment. This could improve their nutritional status for a better clinical outcome.