We report a case of chemotherapy induced acute pan-creatitis in a child with acute lymphoblastic leukaemia. L-asparaginase is the most likely incriminating drug.
Child abuse's trend has been on the rise in Malaysia. Despite strong legislative rules of Child Act 2001, the response of the community is believed to be inadequate. Various factors have been highlighted to ensure effective management of the issue. Child abuse is considered a social emergency due to its complexity and involvement of emotional, family dynamic and domestic instability. The success of the managing these cases largely depends on our urgency and collaborative partnership between multidisciplinary team members, to improve on the welfare of these children, albeit a slower outcome. The community as a whole has to be proactive in reporting hidden cases to avoid mortality or morbidity, as a result of the perpetrator's actions.
This paper looks at some of the problems encountered when cases of abuse and injuries occur in children placed in child care in an urban community in Malaysia. It is based on the study of 37 children referred to our hospital's Child Protection Services over the past four years for injuries and incidents where the child-minders came under suspicion. They constituted 12% of the 285 children referred to the Child Protection Team during that period for evaluation of suspected child abuse. Twenty six children had experienced physical trauma. Fifty eight percent of these were young infants with cranial injuries or limb fractures but no history to explain their occurrence. Four had superficial injuries from accidents secondary to inadequate supervision. The other 11 children had been sexually abused. The perpetrators in 73% of cases were either the husbands or sons of the child-minder. Ninety five percent of the incidents occured at home-based nurseries. Prospects of any punitive measures were remote in most instances due to uncertainty about the perpetrator, or insufficient corroborative evidence required in sexual abuse cases. There were three documented cases where the family refused to lodge a police report or co-operate with investigations out of a reluctance to implicate or offend the child-minders who were mainly neighbours, relatives or friends in 58% of cases. Ninety seven percent of the facilities were unregistered, making surveillance for recurrent incidents and adherence to safety standards difficult. These incidents were almost certainly an underestimate of the problem. Training of child-care providers, improved legislation and enforcement as well as education of parents to choose suitable facilities are issues which need to be tackled.
A retrospective study was conducted to evaluate the magnitude, clinical course and risk factors of cholestasis in surviving very low birth weight (VLBW) infants who received parenteral nutrition at the neonatal intensive care unit (NICU) of Hospital Universiti Kebangsaan Malaysia. Cholestasis was defined as direct hyperbilirubinaemia of >34 mmol/L. Between 1St July 2000 to 31St March 2001, 58 VLBW infants received parenteral nutrition (PN). Forty seven infants survived to discharge. Complete data was obtained from 43 (90.1%) infants. Thirteen (30.2%) of these infants developed cholestasis. The cholestasis persisted beyond the age of 6 weeks in 8 infants and they underwent hepato imino diacetic acid (HIDA) scan. The HIDA scan was reported as normal in 2 infants and inconclusive in 6 infants. Operative cholangiography. (OTC) was then performed in these infants and were all normal. The cholestasis finally resolved in all infants. Analysis of risk factors revealed that duration of assisted ventilation and PN and the presence of patent ductus arteriosus (PDA) had significant correlation with the development of cholestasis. These factors together with lower mean gestational age increased the likelihood of persistent cholestasis beyond 6 weeks of life in these infants. In conclusion cholestasis is common in VLBW infants who received parenteral nutrition. Although the clinical course seems benign, in a significant proportion of these infants it may persist longer and put them at increased risk of requiring invasive investigations.
Chronic granulomatous disease (CGD) is defined as an inherited phagocyte disorder causing defective superoxide generation and intracellular killing. Reduced or missing burst activity of nicotinamide dinucleotide phosphate (NADPH) oxide complex is observed in this inborn defect that usually manifests itself during the first two years of life. It can be inherited either by X-linked inheritance or autosomal recessive inheritance. Most patients with CGD develop failure to thrive, severe bacterial adenitis, abscesses, osteomyelitis or hyperinflammaory manifestations. (Copied from article).
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.
The report describes a pilot study of cognitive-behavioural social skills program conducted on primary school children in Kelantan, Malaysia. Designs: Uncontrolled pre- and post- single design study. Methods: Children aged 9 - 11 referred by school teachers for behavioural problems were put into Petersen’s Stop-Think-Do cognitive-behavioural social skills program for 6 sessions delivered by a clinical psychologist. Main outcome measure was Goodman’s Strengths and Difficulties Questionnaires; to assess child’s emotional and behavioural functioning rated by parents and children themselves. Results: The results showed positive short-term effects of the program at improving some aspects of children’s psychological functioning, however they are not significant. Findings are limited to several issues including subjects’ drop-out, problems in generalizing learnt behaviour, and the use of single outcome measure. Conclusion: Whilst the program could possibly improve children behavioural functioning more dramatically, the implementation of such program in school setting must be organized in full collaboration and assistance from the parents and school administrators.
Objective: To compare the mean duration of diarrhea between children taking yogurt and with the group taking commercial probiotics, containing lactobacillus in acute watery diarrhea. Materials and
Methods: This descriptive study was conducted in the Department of Pediatrics, Services Hospital, Lahore. The duration of this cross sectional study was 6 months, from June 2013 to November 2013. A total of 200 children suffering from acute watery diarrhea were involved in the study after the informed consent under the said hospital. Their demographic information was recorded and later divided in two groups of 100 each; group A received yogurt and group B was given commercial probiotic. The duration of diarrhea was observed in both groups. The results were compared by using independent sample t-test.
Results: The average duration of diarrhea in group A, was 1.98 + 1.31 day while that in group B was 3.09 + 1.64 days. Student's t-test was applied and the difference between the two groups was found to be statistically significant (p< 0.05).
Conclusions: Treatment with yogurt significantly decreases the mean duration of diarrhea as compared to probiotic among patients with acute watery diarrhea.
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.
Respiratory syncytial virus (RSV) is isolated in 15-25% of young Malaysian children with bronchiolitis.',2 Although this observation is consistent with experience reported in other developing nations in the tropics,3•4 it is lower than that of temperate developed nations where RSV is isolated in 60 - 80% of young children with viral bronchiolitis.5,6 The majority of infections are mild, easily cared for at home and only 1% of children with RSV bronchiolitis require in-hospital care.' However, several categories of children have been identified to develop severe RSV bronchiolitis that is asso-ciated with an increased risk of mortality and significant morbidity. This 'high-risk' group includes children who are very young, ex-premature (gestation less than 36 weeks), children with chronic lung disease, congenital heart disease and immunodeficiency, namely, haematological transplant recipients.8'9 It is for this category of children in whom effective therapeutic strategies for the treatment of RSV bronchiolitis are most important.
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.
Lumbar puncture (LP) is an important procedure both for diagnostic and therapeutic purposes. In Kelantan, Malaysia, on many occasions consent for this procedure is not granted by patients or guardians. The aim of this study was to find out the factors that influence the decision to grant or refuse the consent for LP. This was a cross-sectional study in which 86 parents (who agreed or refused to give consent for LP on their child) were interviewed by using a standard questionnaire. A scoring system was used to assess their knowledge about the purpose and technique of the procedure. Consent for LP was granted in 23 and refused in 27 children. The refusal rate was significantly higher when family members other than parents made the decision. The factors which did not play a statistically significant role in decision-making included: age of the child and parents; gender of the patient; number of children in the family; patient's birth order among the siblings; place of residence; monthly income of the family; parents' level of education; and number of days after admission when LP was requested. The factors that positively influenced the decision to give consent included knowledge about the purpose of LP and underlying disease. In order to receive positive consent for LP it is concluded that the parents/guardians and other family members who may influence the decision-making should be explained the role of LP in (1) making diagnosis and (2) choosing right antibiotics for treatment. They should also be informed about the side-effects of antibiotics, which may be used unnecessarily in unconfirmed cases of central nervous system infections.
Intravenous immunoglobulin (IVIG) therapy in Kawasaki disease (KD) has been shown to reduce coronary artery aneurysm by 4-5%. However, we still observed significant number of coronary aneurysm post IVIG in our centre. The objectives of this study were to determine the prevalence of coronary artery abnormality (CAA) and the associated risk factors.
Designs: Retrospective descriptive study. Method: A retrospective study performed on children with KD from 1 st January 2005 to 30 th July 2010. Japanese Ministry of Health criteria were used to classify coronary arteries abnormality. Children with incomplete or atypical KD were excluded. Data were extracted from Pediatric Cardiology Clinical Information System.
Results: A total of 126 KD were diagnosed during the study period with 69% were male and 52.4% were Chinese. The median age of diagnosis was 1.4yr [Q1, 0.6yr Q3, 2.3yr]. Of these 126, 118 (93.7%) received IVIG within 10 days of illness. Ten patients (7.9%) required more than one dose of IVIG. CAA were noted in 28 (22.2%) patients with 21 ectasia, 4 small fusiform, one small saccular and 2 medium fusiform aneurysm. Of these 28, 22 who had IVIG within 10 days of illness (18 ectasia, 3 small and one medium coronary aneurysm) Significant risk factors for CAA were older children (2.3 v s 1.7yr, p=0.03), presentation after 10days of illness (p=0.006) and required more than 2gm/kg of IVIG (p=0.04).
Conclusion: CAA in complete KD treated with IVIG was 22.2% with 5.5% significant aneurysm. Risk factors for coronary abnormality were older children, late presentation and require more of IVIG of than 2gm/kg.
Primary immunodeficiency disease (PID) or inborn error of immunity is a heterogeneous group of inherited diseases affecting the immune system resulting in increased susceptibility to infections, immune dysregulation, autoimmune manifestations, lymphoproliferation and malignancy. Cases of PIDs have been reported in Malaysia since 1977 and the numbers of reported cases steadily increased for the past 30 years with more trained clinical immunologist available, better immunodiagnostic facilities, wider immunoglobulin replacement therapy availability and improved techniques in haematopoietic stem cell transplantation for PIDs. In this article, we highlight some of the limitations and challenges in the diagnosis and therapy of PID, and more recent efforts to establish PID services in Malaysia.
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.
Objectives: Chromosomal abnormalities especially aneuploidies are the most common etiology for pregnancy loss. Trisomy 13, trisomy 18 and trisomy 21 are the most common chromosome autosomal aneuploidies with trisomy 21 (Down syndrome) being the most common chromosomal abnormality among liveborn infants. In previous reports, we noted that the recurrence of these aneuploidies in some families may not occur by chance alone.
Methods: Extraction of relevant data from review of medical case notes of a young couple with two offspring with Down syndrome (DS) and Patau syndrome.
Results: A family history of DS is a predisposing factor for both DS and other types of aneuploidy. Certain instances of non-disjunction error are not random.
Conclusion: As the maternal age was not advanced in both pregnancies, there is a possibility that the recurrent aneuploidy in this family may not be accounted by chance alone. The risk of having subsequent affected pregnancy cannot be ignored in this family and prenatal diagnosis is strongly recommended in the subsequent pregnancy.
Generally, the karyotype profile of Down Syndrome has been reported to be full trisomy 21 in 92% of patients, mosaic trisomy 21 in 4% of patients and translocation involving chromosome 21 in 4% of patients in most of the population groups worldwide. But, karyotype analysis of 149 DS patients at the Human Genome Center, USM, during the past five years revealed that free trisomy accounted for 94.6%, mosaic trisomy 21 for 4.7% and translocation involving chromosome 21 in 0.7% of the Down Syndrome etiology in North East Malaysian population, indicating a low frequency of translocation DS in this region. Here, we report one case of translocation Down Syndrome encountered during karyotype analysis of 149 DS cases. Karyotype showed a robertsonian translocation where an entire extra chromosome 21 was attached to the centromere of one of the chromosome 14, resulting in a derivative chromosome 14 with attached chromosome 21. Karyotype analysis of the parents revealed a normal 46,XY pattern for father and 46,XX pattern for mother indicating that this robertsonian translocation had arisen de novo either prior to or at conception.
Objective: To describe the effect of mycophenolate mofetil in Pakistani children with steroid dependent and steroid resistant nephrotic syndrome. Methods: This is cross sectional retrospective review of 16 patients; 9 boys and 7 girls (11 SD/FRNS and 5 SRNS) for a period of 4.8 years. This study was conducted in Mayo hospital and Fatima Memorial hospital specialist care centre, Lahore involving urban and suburban population. Results: The median age of the group was 4 years (1.6 to 12.6 years). Seven patients had histological diagnosis of MCN, 3 had diffuse mesangial proliferation, one of membranoproliferative glomerulonephritis and 4 had FSGS. Out of 5 SRNS 4 were found to have FSGS and 1 had membranoproliferative glomerulonephritis (MPGN). A total of three patients were completely off steroids and in two patients MMF was also successfully stopped. Number of relapses /patient /year calculated by applying Wilcoxan signed rank test was found to be 4.31 + 0.87(3.00-6.00 /patient/year) before starting MMF, which dropped to 1.12 + 0.718 (0.00- 2.000 /patient/year) after starting MMF, p=0.0001. Reduction in steroid dose from mean of 0.85 + 0.18 mg/kg/day to 0.3mg/kg/day + 1.56 was achieved in 12 months, p
Background: Kawasaki Disease (KD) and acute rheumatic fever are the two leading causes of acquired heart disease in children in the developing countries. Objectives: To determine the epidemiology of KD and its short-term outcome in Malaysian children. Materials & Methods: A retrospective study of patients with a diagnosis of KD at the Kuala Lumpur Hospital from January 1999 to December 2003. Results: 84 patients with KD were seen over the 5 year period. Of these, 52 (61.90%) were male and 32 (38.10%) female. Malays comprised 51 (60.71%), Chinese 30 (35.71%) and Indian 3 (3.57%). Their ages ranged from 2 months to 11 years 1 month old. There were 25 (29.76%) patients less than one year old, 50 (59.52%) aged 1-4 years, 4 (4.76%) aged 5-7 years and 5 (5.95%) were more than 7 years old. Echocardiographic examination during the acute phase showed that 24 patients (28.57%) had coronary artery dilatation; 23 had mild dilatation and one had giant aneurysm involving both coronary arteries. Echocardiographic examination at 8 weeks showed that 5 (21.74%) of the 23 patients with mildly dilated coronary arteries had resolved; the bilateral giant coronary aneurysms remained the same. There was no death due to KD over the 5 year period. Conclusion: KD occurred most commonly in children aged 1-4 years old with a peak at 17 months. There was male preponderance with a male to female ratio of 1.6:1. Despite immunoglobulin therapy, 29% of patients had coronary artery involvementduring the acute phase; 22% of those with mild coronary artery dilatation resolved at 8 weeks after disease onset.
Objectives: This study was conducted with two objectives, i) to assess the prevalence of malnutrition among children and adolescents with learning disability (LD) in Kelantan, a rural state located at the north-eastern region of Peninsular Malaysia; and ii) to examine the associations of participants’ socioeconomic and feeding characteristics with their body mass index (BMI).
Methods: A total of 271 children and adolescents with LD aged between 4 to 19 years old were recruited from 32 community-based rehabilitation centres using purposive sampling method. Standing height and body weight of participants were measured. Socioeconomic and feeding information were obtained from their primary caregivers through interviews using structured questionnaire. Multiple linear regression analyses were performed to examine the associations of socioeconomic and feeding characteristics with participants’ BMI.
Results: The prevalence of underweight among children and adolescents with LD was 22.5%; while another 22.1% of them were overweight and obese. Multiple linear regression models showed that being Down’s syndrome (Adjusted regression coefficient β=2.63, p