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
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.
Objective: This study assessed the pattern of clinical course of hematological disorders in children diagnosed by bone marrow aspiration/biopsy in a tertiary care centre. Setting: The study was conducted at the Department of Pediatrics, Lahore General Hospital, Pakistan. Design: A retrospective descriptive study. Duration of study: Jan 2006 to Dec 2010. Methods: The clinical and laboratory data of 250 patients including complete history, physical examination, investigations and bone marrow examination reports were collected and then analyzed retrospectively. On the basis of these data, relative frequency of different hematological disorders was determined. Results: A total of 250 patients were selected during this study period where their bone marrow was sent for the investigations. Out of these cases, double deficiency anemia was the commonest diagnosis (22%) followed by aplastic anemia (13.6%), megaloblastic anemia (13.2%) and iron deficiency anemia (5.6%). For hematological malignancies, acute lymphoblastic leukemia (ALL) was observed in 27 cases (10.8%) followed by acute myeloid leukemia (AML) in 12 cases (4.8%), lymphoma in 8 cases (3.2%) and chronic myeloid leukemia (CML) in only two cases. Idiopathic thrombocytopenic purpura (ITP) was reported as frequent as 13.2% (33 cases). Conclusion: The pattern of non malignant hematological disorders in children diagnosed by bone marrow aspiration/biopsy was more common than malignant conditions. Double deficiency anemia was the commonest non malignant condition followed by aplastic anemia, idiopathic thrombocytopenic purpura and megaloblastic anemia. ALL was the most common presentation of the hematological malignancy.
Asthma is considered as heterogeneous multidimensional disorder due to variable phenotypic presentation. Phenotype is defined as a cluster of either clinical or pathologic features, which tends to be associated with the understanding the mechanisms of the disease. Asthma is typically characterized by airway inflammation, variable airway obstruction, bronchial hyper-reactivity, smooth muscle hypertrophy and apparent reversible airflow obstruction by bronchodilators. ‘Asthma syndrome’ is a term to describe complex pathophysiology of the condition which is not exclusive to allergen triggered episodes. Diagnosing childhood asthma is difficult, due to the similarity of symptoms and overlapping with other wheezing conditions. The precise mechanism for asthma exacerbation, for each individual phenotype is not fully understood. However, it is influenced by genetic interaction with variety of external environmental stimuli. The current understanding on asthma phenotypes were interpreted based on age of onset, associated triggers, clinical aspects, physiologic parameters and type of inflammation (Table 1). Due to the illdefined of the current phenotypic definition and disagreement among the respirologists, it is becoming a challenge to label specific phenotype with certainty. Accurate definition of each phenotype should therefore be helpful to provide better understanding of different mechanistic pathways and focusing on targeted therapy for individual phenotype. (Copied from article).
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.
Introduction: Parents play an important role in the management of their asthmatic children. Thus the ability of parents to recognise asthma trigger factors are very important.
Objectives: The objectives of this study were to identify the trigger factors that were recognised by parents to cause acute exacerbation in their children and analyse the association of these factors with severity of asthma and parental asthma knowledge.
Methods: Sixty-seven parents were interviewed to identify factors that can exacerbate acute asthmatic attack in their asthmatic children. The factors were then categorised as: infection, exercise, allergen, irritant, emotion and weather. The profiles of children were asthma severity status, duration of asthma, age, frequency of admission and steroid dosage. Parents' profiles were their age, number of asthmatic children and the level of asthma knowledge.
Result: Fifty-six (83.5%) parents identified more than one trigger factor for their children's exacerbation. The commonest frequency was two trigger factors (31.3%). Upper respiratory tract infection (77.6%) was the commonest trigger factor. There was no association between the number of trigger factors with the severity of asthma and level of parental asthma knowledge. The number of trigger factors significantly correlated with asthma duration (r = 0.33, p = 0.006). The asthma severity was associated significantly with weather (p = 0.042) but not with other trigger factors.
Conclusion: The majority of parents recognised more than one trigger factors to cause asthma exacerbation.
An eight-year-old Chinese girl presented with a slowly progressive generalized muscle weakness and wasting, complicated by respiratory failure. She had many hospital admissions requiring ventilator support. Eventually tracheostomy tube was inserted. Initial investigations failed to elicit a diagnosis but a muscle biopsy and histological study confirmed the diagnosis of juvenile acid maltase deficiency.
OBJECTIVE: To assess a better strategy to implement oral iron supplementation in preschool Orang Asli children with high prevalence of iron deficiency, as opposed to the current practice, yet inefficient, of daily oral iron supplementation regime. METHODS: A randomized controlled trial was conducted in preschool children presenting to a remote health center (Klinik Desa Kenang, Sungai Siput, Perak) with iron deficiency state. Oral iron prescribed as a daily unsupervised dose (group A) was compared to a weekly supervised administration (group B) over eight weeks. RESULTS: Before intervention, iron deficiency was prevalent in these children (91.2%). The mean baseline haemoglobin and ferritin levels of group A were 9.9 (+/- 1.1) g/dL and 8.9 (+/- 1.3) mg/L respectively, and that of group B were 9.9 (+/-1.2) g/dL and 9.7 (+/- 1.9) mg/L respectively. After eight weeks of treatment, the mean rise in haemoglobin and ferritin levels of group A were 1.2 (+/- 0.6) g/dL and 18.1 (+/- 15.1) mg/ L respectively, as compared to group B, where the mean rise in haemoglobin and ferritin levels were 1.8 (+/- 0.7) g/dL and 35.2 (+/- 21.8) mg/ L respectively. The differences in the rise of haemoglobin and ferritin levels of the two groups were statistically significant (p
Even though vaccines are effective for prevention of diseases, compliance with recommended schedules of immunisation in early childhood is not always complete. Identification of potential risk factors associated with under-immunisation may help increase vaccination uptake. The objectives of this study were to determine the prevalence and risk factors of under-immunisation in hospitalised preschool children in Universiti Sains Malaysia Hospital (HUSM). Children aged 1 month to 2 years admitted between December 1998 and December 2000 in medical paediatric wards at HUSM were studied. Parents/guardians were interviewed during admission regarding immunisation status and potential risk factors. Children who did not have home-based cards during admission were excluded. Among 401 children, 37.9% had complete immunisation, 43.4% had delayed immunisation and 34.9% had incomplete immunisation. The uptake of BCG, DPT/OPV 1, DPT/OPV2, Hepatitis B3, DPT/OPV3, and measles were 98.5%, 90.7%, 83.2%, 81.4%,79.2% and 72.9% respectively. Vaccines scheduled to be given at later ages were significantly less likely to be taken (p=0.002) compared with vaccines scheduled to be given at an earlier age. Significant independent risk factors for under-immunisation were elderly mothers (3 40 years old) (OR 3.0, 95% CI: 1.2-8.0), low birth weight £2.0 kg (OR 6.2; 95% CI: 1.8-21.4) and presence of chronic illness in the child (OR 4.5; 95% CI: 2.5-7.9). In children whose father's education was low (primary school or no education), low maternal education was significantly associated with lower rate of under-immunisation (p = 0.005, OR 0.11 95%CI 0.02-0.51).
In conclusion this study showed that a large proportion of hospitalised preschool children were under-immunised, and vaccines scheduled to be given at a later age had lower uptakes. The situation can be rectified if every opportunity is taken to immunise all children, who have lapsed in their immunisation schedule.
Heralded by the revelation of the double helical structure of the DNA molecule in 1953, the 21st century is aptly designated the biotechnology century. The 20th century of physics, which saw the transformation of silicon into computing magic, was embraced with enthusiasm by virtually every household. However, unlike her predecessor, the same cannot be said about the advancements in biomedicine.
A Malay baby boy with the femoral-facial syndrome is presented. The phenotype included bilateral femoral hypoplasia, short nose with a broad nasal tip, long philtrum with a thin upper lip, micrognathia, bilateral cryptorchidism and hypoplastic penis.
Four hundred and forty three teachers from eight ran-domly selected primary schools were enrolled in this study which was aimed at determining the knowledge regarding child abuse and neglect among primary school teachers in Kuala Lumpur. The areas that were examined included characteristics of child abusers and abused children. The results showed that the marital status of the teachers did not influence their level of knowledge and there was no significant difference between novice and senior teachers. The most impor-tant finding here was that knowledge regarding sexual and physical abuse was lacking in the teachers surveyed.
Ascaris lumbricoides infection usually results in mild gastrointestinal symptoms. However, heavy worm load infections can cause major complications. In one-third of the latter cases, the worms enter the bile duct causing cholangitis, biliary colic and acute pancreatitis. We describe an eleven-year-old Indian boy who presented with high grade fever, vomiting of worms, jaundice and right hypochondria! pain. Examination revealed an ill, malnourished and jaundiced child. His abdomen was tender with positive Murphy's sign and hepatomegaly. Urgent ultrasound showed hepatomegaly with dilated intrahepatic and common bile ducts (CBD). There were tubular echogenic filling defects with central sonolucency along the long axis of the CBD. He was fasted, started on intravenous fluids and broad spectrum antibiotics. As there was no improvement after 24 hours, endoscopic retrograde cholangio-pancreaticography (ERCP) was performed: it showed worms in the CBD. A stent was inserted to decompress the bile ducts and to ensure biliary drainage. The patient improved, the fever settled, jaundice resolved, serum amylase and transaminase levels normalised. It was planned to remove the stent at a later date.
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.
The problem-based learning (PBL) curriculum, which originated primarily from the Faculty of Health Sciences at McMaster University in 1969, has had a major impact on the thinking and practice in medical education. It is one of the most important developments in the health profession's education in the latter part of the twentieth century) The PBL process incorpo-rates fundamental educational principles such as those derived from adult learning theory' and this gives the PBL approach a greater effective-ness for the acquisition of basic knowledge and clinical skills.
Inborn errors of metabolism (IEM) are a group of disorders that causes abnormal function of biochemical pathways. Archibald Garrod des-cribed the first inborn error of metabolism in 1893. He described alkaptonuria in a patient whose urine turned black on standing and the development of arthritis in adult life.' Subse-quently, Garrod encapsulated the idea of IEM in 1908 with the concept of 'chemical indivi-duality'. Beadle and Tatum proposed the concept of one gene - one enzyme in 1945.2 Phenyl-ketonuria (PKU) was described in 1934 and amongst the first to be recognised as a cause of mental handicap with a biochemical basis.' Effective treatment for PKU with low pheny-lalanine diet was introduced in 1955. Molecular characterisation of genetic defects localised to alleles in various chromosomes were performed in the last two decades
The objective of the study was to determine the prevalence of hypertension in healthy primary school children. A cross-sectional survey by manual blood pressure measurement of healthy children aged 6-12 years attending a national type school was carried out. Children with previous renal, cardiac and endocrine disorders were excluded. Korotkoff 1 represented the systolic blood pressure (SBP) and Korotkoff 5 was taken as the diastolic blood pressure (DBP). Hypertension was defined as the SBP or DBP above the 95th centile according to age group and sex regardless of ethnicity. A total of 1756 children were studied with 895 boy and 861 girls. The results showed that 109 children (6.2%) were found to have hypertension and the incidence was similar in boys and girls (6.4% vs 6.0%, p = 0.69). Fifty-three (48.6%) children had an elevated SBP and the DBP was elevated in 43 (39.4%) children. Only 12 (12.0%) children had both elevated SBP and DBP. Children found to have hypertension were more likely to be Chinese (p = 0.009) and obese (p = 0.04). In conclusion the overall prevalence of hypertension in children aged 6-12 years based on a single blood pressure measurement was 6.2%. Hypertension was more likely to be found in Chinese children and those who were obese.
Helicobacter pylori (HP) was first described in 1983 by Warren and Marshall.' It is a spiral-shaped bacterium measuring 2-4p,m x 0.5-1.0μm. Since it was discovered, the organism has rarely been isolated from sites other than the stomach.2 Available evidence, therefore, points to the human stomach as the normal habitat of this bacterium. The infection is contracted primarily in childhood.' It has been shown that colonisation by HP is rare under the age of five years, but thereafter, it becomes gradually more frequent, and by sixty years of age more than 50% of individuals may be affected
Meconium aspiration syndrome (MAS) frequently occurs in neonates born in thick meconium-stained liquor (TMSL). It continues to be a significant cause of neonatal morbidity and mortality despite aggressive approaches to intervention. This study aims to identify the perinatal characteristics of deliveries with TMSL associated with the development of MAS and to describe the outcome of those babies who developed MAS.
All newborns with TMSL born in Hospital Alor Setar, Kedah from 1 October 1998 to 30 April 1999 were prospectively studied. In the hospital, all newborns with TMSL were routinely admitted to the Special Care Nursery for observation. Relevant data regarding pregnancy, delivery, resuscitation and outcome of newborns with and without MAS were collected and analysed. The incidence of TMSL among hospital deliveries was 2.2% (95% C.I. 1.8-2.5%). Thirty-six percent (95% C.I. 28.4-44.7%) of newborns with TMSL developed MAS. Significant risk factors associated with the development of MAS were non-Malay mothers (p=0.04, OR=3.32), resuscitation by paediatric medical officers (p=0.02, OR=2.84) and direct tracheal suction during resuscitation (p=0.02, OR=2.61). The presence of non-Malay mothers or resuscitation by paediatric medical officers or direct tracheal suction during resuscitation gave a sensitivity of 88.2%, specificity of 36.7%, a positive predictive value of 44.1% and a negative predictive value of 84.6% for the development of MAS. Asphyxia, pneumothorax and persistent pulmonary hyper-tension occurred in 25.5%, 7.8% and 5.9% of the babies with MAS respectively. Mechanical ventilation was required in 51% and the mortality rate was 3.9%. In conclusion, the incidence of TMSL and MAS and the mortality rate of neonates with MAS were comparable with that in developed countries. The presence of any one of the significant risk factors had high sensitivity but low specificity for the development of MAS. Newborns were unlikely to develop MAS if all these risk factors were absent.
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.