Life (QOL) of patients with childhood leukaemias presenting at the Hospital Universiti Kebangsaan Malaysia (HUKM). The objectives of this pilot study were 1) To assess the feasibility and applicability of assessing quality of life in leukaemia patients using the adult-based SF-36 questionnaire. 2) To compare the differences of QOL scores among patients based on gender and treatment status. The Short-Form-36 Health Survey (SF36) was used. The items in SF-36 were drawn from the original 245-item MOS questionnaire, which includes multi-item scales that measured the eight dimensions of quality of life namely physical function, role limitations (physical problems), bodily pain, general health, vitality, social functioning, role limitations (emotional problems) and mental health. Patients with acute leukaemias were chosen to participate in the study. Twenty-eight patients were recruited, twelve were males and 16 were females. Ten patients had completed therapy whilst 18 others were still undergoing treatment. The results showed that those patients off treatment have a better quality of life than those on chemotherapy. However, this was only significant with respect to the role limitation pertaining to physical problems. Although the use of the SF-36 was feasible, there were limitations especially in the younger age group.
Significant advances in perinatology and neonatology in the last decade have resulted in increased survival of extremely premature infants.' Survival rates at 25 and 26 weeks of gestation age ranging from 60% to 82% and from 75% to 93%, respectively, have been reported.' In Malaysia, the survival rates among premature very low birth weight infants (< 1500 g) were reported to be between 69% and 78%.2,3 Such improvements of survival have been attributed to the advances in the management of respiratory disease and intra-ventricular haemorrahge in the premature infants.',2 Thus, attention have recently been focused on the need to secure adequate nutrient intake of these premature infants. Parenteral nutrition has often been used to manage the transition between transplacental nutrition in-utero and post-natal enteral nutrition, but is associated with cholestasis and sepsis.4 However, the ability to deliver nutrition is limited not only by immature absorptive or digestive function but by inadequate motor activity. Gastroesophageal efflux (GER) and feeding intolerance are the major gastro-enterological problems of the premature neonates.
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.
We report a rare case of a newborn baby girl who was delivered at 36 weeks of gestation with birth weight of 2680 grams and has ankyloblepharon filiforme adnatum with cleft lip and palate diagnosed at birth. The baby was managed with simple procedures of eyelid bands separation. We also reviewed the embryology of eyelid and its relation with ankyloblepharon formation and discussed the various syndromes and abnormality which may be associated with this eyelid abnormality. The four types of ankyloblepharon which was described by Rosenman and colleague is presented.
Introduction: There are many factors that determine the survival of patients with VSD. Among these include size of VSD, position, pulmonary hypertension, bacterial sepsis, valvular involvement, associated anomalies with VSD, associated syndromes and age at first diagnosis. There has been no published local data as far as we know and this information will be useful especially for consultation with parents. Even though VSD in general has a good prognosis, whenever they have added risks for example pulmonary hypertension then they are at risk of further morbidity and mortality. Objective: To determine the factors that are associated with survival of patients with VSD. Design: Retrospective cohort. Materials and methods: All cases of isolated VSDs admitted to HUSM from 1996 to 2003 were reviewed. Results: Univariate Cox regression of survival time of patients with VSDs revealed that 4 factors had prognostic significance namely bacterial sepsis (HR= 287.7, 95% CI 51.1, 1618.5, P < 0.001), Down syndrome (HR = 14.89, 95% CI 3.00, 73.92, P = 0.001), pulmonary hypertension (HR=14.58, 95% CI 1.69, 125.7, P=0.015) and large VSDs (HR=8.23, 95% CI 1.5, 45, P=0.015). Bacterial sepsis was the only significant prognostic factors for the survival of patients with VSDs using the multivariate Cox proportional hazard model. Conclusion: Bacterial sepsis, pulmonary hypertension, large VSD and Down syndrome were the significant prognostic factors from Univariate Cox analysis, however bacterial sepsis was the only significant prognostic factor from Multivariate Cox analysis.
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.
Vaccines, used appropriately and efficiently, have changed the landscape of infectious diseases. Poliomyelitis is almost completely eliminated globally. In many industrialised countries, there has been over 99 percent reduction in incidence of diphtheria, tetanus, measles, mumps, rubella, Haemophilus in-fluenzae b meningitis and over 97 percent reduction in whooping cough.',2Unlike anti-biotics, most vaccines have remained equally effective despite years of continuous usage.
Neuroblastoma is usually presented with abdominal distension. However, central nervous system manifestations of neuroblastoma are uncommon. In this case report, patient presented with uncommon presentation of neuroblastoma and the diagnostic dilemma.
Neonatal malaria may be overlooked likely due to its non-specific features and low prevalence in Malaysia. In this case report, we detail a case of neonatal malaria in an 18-day old baby girl of Myanmar origin who presented with 6 days of intermittent fever but was otherwise well. Initially, she was treated as neonatal sepsis. She then developed thrombocytopaenia and severe anaemia with persistent spikes of temperature. This prompted a series of investigations and multiple changes of antibiotics. The diagnosis of neonatal malaria surfaced when her peripheral blood film incidentally revealed the presence of Plasmodium vivax parasites. Peripheral blood smears are simple and inexpensive. Therefore practising especially in endemic areas for malaria, we need to consider this diagnosis when dealing with neonatal sepsis that does not respond to standard treatment.
Lymphangiomas are hamartomatous congenital malformations of the lymphatic system that usually involve subcutaneous tissues of cervico-facial region. Rarely, it can be found in subcutaneous tissue of proximal extremities, the buttocks and the trunk. Magnetic Resonance Imaging (MRI) is the best modality to assess the tumor specification and extension. We report a case of lymphangioma at a rare site with its radiological features and patient responsed to the sclerosant therapy.
Progression to AIDS is more rapid in HIV-infected children. Objective: Our study aimed to investigate the effect of HIV status disclosure in children in terms of their perception of the illness, knowledge on their disease and medications and the overall impact on their quality of life.
Methods: A qualitative study was conducted from 1st June 2017 till 8th September 2017 involving face-to-face interviews with HIV-positive children receiving combination anti-retroviral therapy (cART) under paediatric HIV clinic follow-up, Hospital Tuanku Fauziah, Kangar, Perlis. Transcribed interview dialogues were subsequently coded for analysis.
Results: Data were collected from 6 out of 8 HIV-positive children on cART in Hospital Tuanku Fauziah, Perlis. Participants’ mean age was 12.2 years (standard deviation [SD] 2.11); 3 with disease disclosure. Disease-disclosed participants with ironically poor knowledge on their disease emerged as the key theme in the study. Disease-disclosed participants were also noted to be more withdrawn with very few friends, if any. Disease disclosure status did not affect the patients’ knowledge and compliance to medication.
Conclusion: Assessment of patients’ understanding after disease-disclosure session is crucial to encourage therapy adherence and prevention of future transmission. Re-appraisal of the healthcare policy with regards to holistic management of children living with HIV is warranted to attain positive social and developmental goal throughout their lives.
Study site: paediatric HIV clinic follow-up, Hospital Tuanku Fauziah, Kangar, Perlis
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.
Introduction and Objective: Pneumococcal disease is a leading cause of morbidity and mortality worldwide. There were limited publications on invasive pneumococcal infection (IPD) in Malaysia. The aim of this study is to describe restrospectively cases of IPD in hospitalised children of less than 12 years old and highlighting the unusual cases.
Methodology: A retrospective review of children with IPD from March 2002 to November 2005 at a tertiary paediatric hospital. IPD cases were defined as isolates of Streptococcus pneumoniae from a normally sterile body fluid site.
Results: Twenty-four patients were identified with a male preponderance. Two-thirds of patients were below 1-year-old; with three cases presenting in the premature newborn. Thirty-seven percent of cases had underlying conditions. Sepsis and pneumonia were the commonest manifestation, followed by meningitis. The unusual manifestations were in a form of postinfectious glomerulonephritis and overwhelming purpura fulminans. There were two mortalities; both infants had meningitis. Antibiotic susceptibility pattern showed that more than half of the isolates were sensitive towards penicillin and erythromycin. Penicillin resistance was found in 6 (25%) isolates. Conclusion: IPD results in significant morbidity and mortality, especially in young children below 2 years of age and justifies further evaluation of preventive strategies including the implementation of pneumococcal vaccine in the national immunisation programme.
Music therapy is rarely available within the medical services in Malaysia. Hospitalized children experience a great deal of stress, anxiety, and pain due to unfamiliar environment, separation from the family, a variety of medical procedures and treatments, and the uncertainties of the illnesses. Music is often used to cope with both physical and psychological distress clinically because of its pervasiveness and familiarity. The purpose of this article is to elaborate the effectiveness of music therapy in addressing both physical and psychosocial needs of hospitalized children. The three case studies demonstrated how a variety of music therapy interventions are used to facilitate individualized goals and to improve the quality of life.
Introduction: Vaccine-preventable diseases such as pertussis are re-emerging in Malaysia during recent years.
Objective: This research aims to study the local incidence of clinical pertussis among paediatric patients admitted to Hospital Tuanku Fauziah, Perlis over two-year period.
Method: A cross-sectional study was designed in Department of Paediatrics, Hospital Tuanku Fauziah, Perlis from 1 January 2013 till 31 January 2015. Data were collected from medical and laboratory record of cases admitted for clinical pertussis. Analyses for descriptive data were done using frequency and percentage; Pearson chi-square or Fisher exact was used to test association.
Results: 81 cases of clinical pertussis were included in the study. Out of this, there were a total of 28 confirmed cases of pertussis. There was a steady increment in the incidence of pertussis throughout the study period. Cyanosis emerged as the only clinical sign significantly associated with pertussis (p = 0.011). Majority of the confirmed pertussis cases were too young to be immunized (n = 13, 46.4%).
Conclusion: Reappraisal of local health system to strengthen herd immunity in the community is warranted to control disease spread.
Goltz syndrome or Focal Dermal Hypoplasia (FDH) is an uncommon genetically inherited disorder characterized by distinctive skin abnormalities and a wide variety of multisystem defects which was first described by Goltz (an American dermatologist) in 1962. About 200- 300 cases have been reported worldwide. FDH can be inherited in an X-linked dominant manner with in-utero lethality in males. Majority of the cases are sporadic with new mutations arising in the embryo and not inherited from a parent. Approximately 10% of cases occur in males; postzygotic somatic mosaicism accounts for the findings in these affected males. FDH is caused by abnormalities or mutations at the PORCN gene in the X chromosome. We report a case of FDH with characteristic skin lesions as well as multiple digital anomalies - oligodactyly, syndactyly and ectrodactyly.(Copied from article)
Neonatal Central Diabetes Insipidus (CDI) is extremely rare and its causes include infection, trauma, hemorrhage or tumor. A high index of suspicion is necessary as early treatment is required to prevent further complications. We report a case of Neonatal CDI as a complication of a Serratia brain abscess.(Copied from article)
HbE/β-thalassemia is the most common severe form of thalassemia particularly in SEA region including Malaysia and globally, it comprised of a significant severe form of β-thalassemia disorder. It has various clinical manifestations ranging from very mild anemia to severe manifestation similar to beta thalassemia major. Many different syndromes are observed in HbE/β-thalassemia. Several genetic modifiers have been reported to play important role in contributing to phenotypic variability. The true reasons underlying this phenotypic variability remain unknown. The most reliable predictive factor of the disease phenotype is the nature of the beta globin gene mutation itself. However, the degree of severity is also believed to be affected by other genetic modifiers. For instance, high HbF level ameliorates the clinical severity of β thalassemia patients. Therefore, identification of these genetic modifiers is very important. The association of severe clinical manifestation and the specific β-globin gene mutation has been known. But the wide scope and other potential predictors have been only recently appreciated. This review therefore aimed to reveal the potential genetic modifiers of HbE/βthalassemia patients based on the previous reported studies. A better understanding on the mechanisms underlying the variety of phenotypes of this disease may lead to the direction for a better future management plans. This also promotes “personalized medicine” in patient care.
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)
Background: Pneumonia is defined as the inflammation of parenchyma of the lung. It is a substantial cause of morbidity and mortality in childhood throughout the world. The incidence of pneumonia in children under the age of five years is 0.29 episodes per child-year, which equates 151.8 million cases annually in developing countries. Objective: To determine the risk factors for complicated pneumonia.
Material and Methods: This case-control study conducted in Medical Unit III, The Children’s Hospital & Institute of Child Health Lahore. Out of total of 180 cases of pneumonia, 100 were labeled as complicated pneumonia (case) and 80 were labeled as uncomplicated pneumonia (control). Complicated pneumonia included pneumonia with associated complications. Detail history was taken in both groups and recorded on predesigned proforma. Data was analyzed by SPSS 20. Quantitative risk factors like child age, maternal age and father age were analyzed by mean and standard deviation. However qualitative risk factors like method of feeding, malnutrition, immunization, anaemia, and non-vaccination were analyzed by applying chi-square test and finding odd ratios.
Results: Most significant risk factors associated with complicated pneumonia included younger age, maternal and father education, rural area, malnutrition, anaemia, rickets, birth problems, admission during neonatal life due to pneumonia, bottle feeding, nonvaccination, referral and delayed in presentation (p