Choanal atresia is an uncommon malformation of the upper airway. Bilateral choanal atresia usually detected earlier as it is often associated with severe asphyxia immediately after birth. The diagnosis is often delayed in unilateral type, and mostly presented with unilateral chronic nasal discharge. This condition is rarely encountered but should be considered as a possible differential diagnosis of persistent nasal discharge. We present a case of unilateral congenital choanal atresia in a 4-month-old baby girl presented with persistent unilateral nasal discharge. Diagnostic investigation and possible surgical treatment techniques were discussed.
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.
Inherited thalassaemia disease is commonly found in many countries of the world. Care of the disease requires comprehensive management strategies comprising of clinical management of both transfusion dependant thalassaemia (TDT) and non-transfusion dependant thalassaemia (NTDT). It also includes preventive measures such as screening programmes and genetic counseling in order to contain the genetic transmission. At the moment, the only cure is through haematopoeitic stem cell transplant (HSCT). This report illustrates thalassaemia disease prevalence in the Terengganu state and the evolution of care since National Thalassaemia Programme was launched in 2009.
Background: This paper aims to report a rare case of congenital giant cell fibroma (GCF). To the best of our knowledge, this is the first reported case of GCF in new-born. Case Report: A healthy one-month-old baby boy was referred to Department of Paediatric Dentistry for management of swelling on the upper left alveolar region which presented since birth. Clinical examination demonstrated a well define firm swelling over the upper left alveolar ridge, otherwise the swelling was asymptomatic. Patient was monitored periodically. At 1 year and 9 months of age, there were episodes of ulcerations and bleeding from the lesion as a result of trauma from eruption of opposing teeth. Surgical excision of the lesion was carried out under general anaesthesia. The histopathological examination (HPE) report interpreted the lesion as GCF. Conclusion: GCF is rare fibrous lesion that could be diagnosed only on HPE. Although it is an uncommon congenital lesion, GCF should be considered as one of differential diagnosis of swelling over the gingiva.
Aspiration of a foreign body is rare in school-age children. This reports the 21-day journey of an 8-year-old girl who had a foreign body aspiration. She presented to our hospital after five days of respiratory distress. She subsequently required mechanical ventilation and was supported with triple inotropes. After 18 days, a foreign body was removed via rigid bronchoscopy, followed by a rapid recovery of the patient.
Purpose: We aim to describe the clinical profile and visual outcome of paediatric patients who underwent cataract surgery in a tertiary ophthalmology referral centre in West Malaysia from 2013 to 2018. Methods: This is a retrospective review of all paediatric patients who underwent cataract surgery in our centre from 2013-2018. Results: A total of 35 eyes from 23 patients were included. There were 10 (43.5%) female and 13 (56.5%) male patients. Twelve patients (24 eyes) had bilateral cataract while 11 patients had unilateral cataract. Sixteen (45.7%) eyes had congenital cataract, followed by ectopia lentis (n=10, 28.6%), traumatic cataract (n=8, 22.9%) and steroid induced cataract (n=1, 2.8%). Three types of intraocular lens (IOL) were implanted: which were posterior chamber IOL (n=22, 62.9%), iris claw IOL (n=12, 34.3%) and scleral-fixated IOL (n=1, 2.8%). Majority of eyes (n=28, 80%) had primary IOL implantation. Twenty-five (71.4%) eyes achieved best corrected visual acuity (BCVA) of 6/12 and better at 6 months post-IOL implantation. There was no statistically significant difference in the BCVA at 6 months post-IOL implantation among the different cataract aetiology, primary or secondary IOL implantation and types of IOL implant. Eight (22.9%) eyes developed post-operative complications, which included posterior capsular opacification (PCO) (n=6, 17.1%), IOL decentration (n=4, 11.4%) and glaucoma (n=1, 2.8%). Nineteen (82.6%) patients required glasses for visual rehabilitation. Conclusion: Majority of the paediatric cataract patients achieved BCVA of 6/12 or better at 6 months post-IOL implantation. The visual outcome among the different cataract aetiology, primary or secondary IOL implantation and types of IOL implanted were similar. PCO was the most common post-operative complication.
Continuous ketamine infusion has been used successfully to treat severe refractory bronchospasm in children requiring mechanical ventilation in the intensive care setting. One of the common side effects known is emergence delirium. There is no standardized treatment for the ketamine-induced emergence delirium although benzodiazepine, haloperidol and dexmedetomidine have been reported to be effective in the past. A 7-year-old girl admitted to a tertiary hospital for life-threatening asthma requiring immediate intubation and ventilation. Ketamine was used as sedative and bronchodilator in anticipation of her challenging ventilation strategy. She was successfully extubated on day 8 of admission, however, she developed symptoms associated with the delirium. Successful reversal of the symptoms was achieved after 48-hour use of low dose intravenous midazolam. This was the first case reported on the reversal of ketamine-induced emergence phenomenon using low dose intravenous midazolam infusion.
Objective: This study aimed to determine the prevalence of anaemia in children aged six months to fifteen years old treated in a single centre from 2008 to 2018. Methods: A retrospective study was conducted among 274 children age six months to fifteen years old, treated in Advanced Medical and Dental Institute (AMDI), Universiti Sains Malaysia (USM) from 2008 to 2018. The data was obtained from computerised hospital data (CARE2X), Laboratory Information System (LIS) or medical record. Parents of the eligible participants were called for any incomplete data and verbal consents were obtained. Descriptive analysis was conducted to determine the prevalence whereas the relationship between independent variables with types of anaemia were examined using simple logistic regression. Results: The prevalence of anaemia was 22.3%. Among the anaemic children, the predominant morphology form was hypochromic microcytic anaemia (82%) followed with normochromic normocytic anaemia (18%). Iron deficiency anaemia (IDA) was found to be the commonest cause (24%) of hypochromic microcytic anaemia, followed by IDA with concomitant thalassaemia (14%) and thalassaemia alone (8%). In simple logistic regression analysis, no significant association was found. Conclusions: The prevalence of anaemia was 22.3%, which is considered as moderate public health problem according to WHO. Hypochromic microcytic anaemia was the predominant red cell morphology (82%) and IDA was the commonest causes (24%).
The causes of neonatal hypoxemia are usually either pulmonary or cardiac pathologies. We report a case of a 2-month-old boy with oxygen dependency since birth. He would desaturate down to 88-90% whenever supplemental oxygen was weaned off. Initial screening echocardiograms described only a small atrial septal defect, thus lung disease was thought to be the aetiology. Eventually, a detailed echocardiogram and CT angiography revealed anomalous right superior vena cava (RSVC) draining into the left atrium (LA). Echocardiography with bubble study injected via the upper extremities showed brisk filling of “bubbles” in the left heart, confirming fixed right-to-left shunting. Anomalous RSVC drainage into the LA is an extremely rare form of anomalous systemic venous drainage whereby unexplained hypoxemia is present and is sometimes the only clinical manifestation. Surgical correction of this anomaly is indicated to prevent complications of cyanosis and risk of systemic embolization. The surgery generally carries low risk and is associated with good long-term prognosis.
Neurofibromatosis Type 1 (NF1), also known as von Recklinghausen’s disease is a multisystem genetic disorder that occurs with an incidence of 1 in 4000 live births. Plexiform nmeurofibroma (PN) is a rare important variant of NF1, seen in 5 to 15% of cases. We report a 7-year-old boy, without family history of neurofibromatosis presented with left neck swelling associated with multiple café au lait spots for one-year duration. Computed tomography of head and neck revealed an extensive soft tissue lesion involving the left parapharyngeal, carotid space and parotid space encasing the left internal and common carotid artery until its origin at the arch of aorta with erosion of the basiocciput. He underwent transcervical excision of the lesion, which was confirmed histopathologically as PN.
Childhood cancer is the number one cause of death by illness in children. However, despite being such a deadly disease, survival from childhood cancer is high with cancer treatment. Unfortunately, undergoing cancer treatment can result in many treatments related side-effects. Daily self-care such as maintaining a balanced diet, drinking enough fluids, practicing proper hygiene and getting enough rest, can be effective in lowering the severity of these symptoms. It is found that existing interventions for cancer patients do not target treatment adherence in children. Therefore, the aim of the research is to design an intervention which is able to motivate children to adhere to treatment and encourage daily self-care. To enhance the design of such an intervention, the article explores the potential of virtual pet games as a health intervention, and the use of the Protection Motivation Theory and Social Learning Theory’s observational learning in increasing the intervention’s effectiveness. Finally, the design considerations, game objectives and overall conceptual architecture of the proposed intervention are discussed. A conceptual comparison is also made between the proposed game and existing serious games for cancer patients. Future work is required to develop this design into a working prototype, and to evaluate its effectiveness on improving treatment adherence and promoting daily self-care with childhood cancer patients.
Epigenetics is the study of heritable changes in gene expression that do not involve changes to the underlying DNA sequence. It is a change in phenotype without changing in genotype which in turn affects how cellsread the genes. The epigenetic change is a regular occurrence but can also be influenced by several factors including age, environment, lifestyle, and disease state. It may have damaging effects that result in diseases like cancer. At least three systems including DNA methylation, histone modification and RNA-associated gene silencing are currently considered to initiate and sustain epigenetic change. New and ongoing research is continuously uncovering the role of epigenetics in a variety of diseases including in childhood solid cancer such as Ewing sarcoma, neuroblastoma, Wilms tumours, brain tumours and rhabdomyosarcoma. A better understanding of epigenetic changes in childhood cancers can guide towards future therapy and diagnosis.
Objective: The present study examined the sensitivity and specificity of M-CHAT-Malay version [M-CHAT(MV)] to discriminate ASD from other developmental-behavioural disorders. Methods: This study was carried out in the Child Development Centre at a tertiary referral centre. Parents of 130 children aged 18–60 months, referred for developmental-behavioural disorders were asked to complete M-CHAT(MV). A child was considered to have ASD ifthey failed any 3 of the 23 total items or 2 or more of the 6 critical items. Results: Looking at the total items, M-CHAT(MV) has a good sensitivity (88.9%) to differentiate between ASD and other developmental-behavioural disorders, although specificity was only 47.8%. However, the critical items only has sensitivity of 71.4% and specificity of 77.6%. Sensitivity for children aged 49–60 months old was lower (80.0%) compared to those in the younger age group (100.0% and 90.3% for those aged 25-36 months and 37–48 months respectively). Based on the ROC curve, the optimal criteria to detect ASD was failing 1 out of 6 critical items or 3 out of 23 total items. Conclusion: M-CHAT(MV) is a good screening tool in differentiating ASD from other developmental-behavioural disorders although the critical items’ criteria may need to be lowered to improve its sensitivity in selected cohorts.
Background:Recent retrospective studies suggest irrelevance of urine screening for neonate with prolonged jaundice. We re-evaluated the incidence of urinary tract infection (UTI) among these infants, their renal outcome and evaluated the cost incurred. Methods: This is a prospective cohort study. Asymptomatic, prolonged jaundiced infants with unconjugated hyperbilirubinemia were screened for evidence of UTI as per local guidelines. Infants with pyuria would have urine sent for culture and sensitivity. Unit cost was referenced from hospital purchase. Statistical analysis was performed using SPSS 24.0. Results:A total of 291 cases were analyzed. Majority were term infants (93.8%). The commonest cause of prolonged jaundice was breast milk jaundice, hence an incidence rate of 0.34%. Only one infant persistently showed single uropathogen on urinary culture with concurrent pyuria. Urinary structures were normal on ultrasonography and there was no evidence of renal cortical scarring. No recurrence of UTI documented in the first year of life. Each “clean-catch” urinalysis costed RM7. This unit cost escalated to RM37 for catheterized sample. A negative urine culture costed RM28 while a positive culture twice this price. The average cost effectiveness ratio (ACER) in this study was RM5856.56 per detection of case. Conclusion: Incidence of UTI is low. In our study, an undesirable outcome is negligible. Unnecessary parental anxiety from the potentially laborious procedure could be avoided. This study refutes previous literature to include such screening in prolonged jaundice as this may well be irrelevant.
Introduction: School refusal is a common cause of chronically poor school attendance. The basis of school refusal behaviour can be grouped into the symptomatic and functional models, the operant conditioning model and the cognitive model. Objective: This paper is aimed to discuss the various systems and models which explain the school refusal behaviour. Method: An initial search of published literature in English Language was performed on the online databases including Google Scholar, Science Direct, EBSCOhost, and Proquest with the main keywords were School Refusal and Self–efficacy. Further sources were identified after consulting the original author for recommendations, and references within the literature retrieved in the initial search. Results: Out of 57 articles found, 41 were used in this review. Development and maintenance of school refusal involve heterogeneous risk factors and processes. The existing theoretical basis of school refusal behaviour can be grouped into symptomatic models, the operant conditioning model and the cognitive model. Conclusion: School refusal depicts a symptom, heralding the need for early intervention before development of a more pervasive mental health disorder. Where the problem had persisted for months to years, the child or adolescent would commonly present with coexisting anxiety or depressive disorders.