BACKGROUND: One of the most commonly used method for dental age assessment is the method reported by Demirjian and coworkers in 1973. It was later modified by Willems and coworkers whereby they “performed a weighted ANOVA” in order to adapt the scoring system.
AIM: To evaluate the applicability of Demirjian and Willems methods for dental age estimation for Malaysian children and to correlate the accuracy of the findings with the chronology of tooth development of premolars and second molars.
MATERIALS AND METHODS: A total of 991 dental panoramic radiographs of 5-15-year-old Malaysian children were included in the study. The mean Demirjian and Willems estimated ages were compared to the mean chronological age.
RESULTS: The mean chronological age of the sample was 10.1±2.8 and 9.9±3.0 years for males and females respectively. Using the Demirjian method, the mean estimated dental age was 10.8±2.9 years for males and 10.5±2.9 years for females. For Willems method, the mean estimated age was 10.3±2.8 years males and 10.0±3.0 years respectively.
CONCLUSIONS: Willems method was more applicable for estimating dental age for Malaysian children. Overestimation in Demirjian method could be due to advanced development of second bicuspids and molars.
Mucopolysaccharidoses (MPS) are a group of inherited disorders caused by the deficiency of specific lysosomal enzymes involved in glycosaminoglycans (GAGs) degradation. Currently, there are 11 enzyme deficiencies resulting in seven distinct MPS clinical syndromes and their subtypes. Different MPS syndromes cannot be clearly distinguished clinically due to overlapping signs and symptoms. Measurement of GAGs content in urine and separation of GAGs using high-resolution electrophoresis (HRE) are very useful initial screening tests for isotyping of MPS before specific enzyme diagnostics. In this study, we measured total urinary GAGs by a method using dimethylmethylene blue (DMB), and followed by isolation and separation of GAGs using high resolution electrophoresis (HRE) technique. Of 760 urine samples analyzed, 40 have abnormal GAGs HRE patterns. Thirty-five of these 40 cases have elevated urinary GAGs levels as well. These abnormal HRE patterns could be classified into 4 patterns: Pattern A (elevated DS and HS; suggestive of MPS I, II or VII; 16 cases), Pattern B (elevated HS and CS; suggestive of MPS III; 17 cases), and Pattern C (elevated KS and CS; suggestive of MPS IV, 5 cases), and Pattern D (elevated DS; suggestive of MPS VI; 2 cases). Based on the GAGs HRE pattern and a few discriminating clinical signs, we performed selective enzymatic investigation in 16 cases. In all except one case with MPS VII, the enzymatic diagnosis correlated well with the provisional MPS type as suggested by the abnormal HRE pattern. Our results showed that GAGs HRE is a useful, inexpensive and practical first-line screening test when MPS is suspected clinically, and it provides an important guide to further enzymatic studies on a selective basis.
Malay is a consistent alphabetic orthography with complex syllable structures. The focus of this research was to investigate word recognition performance in order to inform reading interventions for low-progress early readers. Forty-six Grade 1 students were sampled and 11 were identified as low-progress readers. The results indicated that both syllable awareness and phoneme blending were significant predictors of word recognition, suggesting that both syllable and phonemic grain-sizes are important in Malay word recognition. Item analysis revealed a hierarchical pattern of difficulty based on the syllable and the phonic structure of the words. Error analysis identified the sources of errors to be errors due to inefficient syllable segmentation, oversimplification of syllables, insufficient grapheme-phoneme knowledge and inefficient phonemic code assembly. Evidence also suggests that direct instruction in syllable segmentation, phonemic awareness and grapheme-phoneme correspondence is necessary for low-progress readers to acquire word recognition skills. Finally, a logical sequence to teach grapheme-phoneme decoding in Malay is suggested.
Idiopathic myelofibrosis occurs predominantly in older adults. It is very rarely seen in children. We describe a 3-year-old girl with Down's syndrome who presented with recurrent chest infections associated with anaemia and easy bruising. There was mild hepatosplenomegaly. Full blood picture revealed pancytopaenia with leucoerythroblastosis with absence of circulating blast cells. Repeated attempts at bone marrow aspiration and trephine biopsy were unsuccessful. A trephine biopsy from the tibia showed depressed myelopoiesis and erythropoiesis, megakaryocytes with atypical morphology and increased bone marrow reticulin fibres, findings compatible with idiopathic myelofibrosis. She was treated symptomatically as she was clinically stable. Review of the English literature online yielded 46 reported cases of childhood idiopathic myelofibrosis with variable outcome from spontaneous remission to an indolent course with shortened survival. 6 cases evolved to another malignancy. 5 cases were associated with Down's syndrome.
This study aims to describe the epidemiology of ocular burns and related injuries due to fireworks during the Aidil Fitri celebration on the East Coast of the Peninsular Malaysia. A prospective analysis of all patients with ocular burns and related injuries due to fireworks attended three tertiary hospitals in East Coast of Peninsular Malaysia during Aidil Fitri, which was from 10 September to 17 October 2008. We observed the demographic and injury characteristics, extent of the injuries and presenting visual symptoms. Thirty patients with 34 eyes with ocular burns and related injuries were identified. Patients ranged between 2 and 43 years of age. 70.00% of the patients were 12 years old and below. Of the 30 patients, 29 (96.67%) were male and the left eye was affected in 14 (46.70%). Thermal injuries accounted for 60.0% of the injuries whereas 40.0% were due to exploding firework. Burns to the eyelid and cornea (35.29%) were the most common injuries noted. Fifteen patients (50%) were bystanders whilst a majority of patients (96.67%) had no eye protection. At presentation, 15 (44.12%) eyes had a visual acuity of 6/6 to 6/9 in the injured eyes, while 3 out of 34 eyes (8.82%) had vision acuity 6/60 or worse. In conclusion, fireworks cause serious preventable ocular burns and related injuries especially in children who are the most affected age group. It affects mainly eyelid and anterior segment structures which result in moderate visual loss on presentation. Health education, public awareness and tighter legislation are essential preventive measures to limit the effect of fireworks to the public.
A cross sectional study of thoracic pedicle morphometry in the immature spine of Malaysian population using reformatted computed tomographic (CT) images.
Acute sinusitis is most often a mild self-limiting disease. However, it may progress into severe and life threatening complications. One of the commonest being orbital complication of which visual loss is a direct consequence. In this 10 year retrospective study, the nature of orbital complication, clinical presentation and treatment modalities and outcome seen in children with acute sinusitis in a tertiary referral institute were reviewed. Of six patients, there was a case of preseptal cellulitis, 4 cases of subperiosteal abscess and one case of orbital abscess. Periorbital swelling was a common presenting feature. In 5 cases this was associated with proptosis with one case of impending optic nerve compression. The value of computed tomography and opthalmological examination as a component in the management plan is highlighted. All patients were treated with intravenous antibiotics but evidence of abscess collection warranted urgent surgical drainage in 5 patients, 3 being endoscopic drainage while external approach was done for the remaining 2 patients. Thus a child exhibiting orbital complication of acute sinusitis, prompt diagnosis and treatment is essential in obtaining the best outcome for the child.
We describe a chromosome 6 uniparental disomy (UPD6) in a boy, discovered during a screening for the genetic cause of cleft lip and palate. In the medical literature, almost all documented cases of UPD6 are paternal in origin, and only four were maternal. We present here a report of complete maternal chromosome 6 uniparental heterodisomy. Haplotype analysis was performed using highly polymorphic short tandem repeat (STR) markers that span both arms of chromosome 6. Analysis of these markers revealed the presence of two maternal alleles but no paternal allele, indicating an instance of maternal uniparental heterodisomy. Chromosome analysis of peripheral blood lymphocytes confirmed a normal male karyotype. Advanced maternal age at the time of the infant's birth and heterodisomy of markers around the centromere favors a meiosis-I error. No specific phenotype has been reported for maternal UPD6. Therefore, the cleft lip and palate in the present case probably occurred due to other risk factors. This report provides further evidence that maternal UPD6 has no specific clinical consequences and adds to the collective knowledge of this rare chromosomal finding.
We report the biochemical profiling in two siblings with mitochondrial 2-methylacetoacetyl-CoA thiolase deficiency. Organic aciduria typical of this rare inborn error metabolism was found when the elder sibling presented with an episode of severe ketoacidosis at 20 months of age, which consisted of excessive excretion of ketones, tiglylglycine, 2-methyl-3-hydroxybutyrate, and 2-methylacetoacetate. Blood acylcarnitiness profile showed elevation of C5OH-carnitine, which represents 2-methyl-3-hydroxybutyrylcarnitine. A similar biochemical profile was identified in the younger sibling during screening although he had only mild clinical symptoms. Both patients reported a favourable outcome on follow-up.
We aimed to determine the epidemiology and burden of rotavirus (RV) gastroenteritis (GE) in children requiring hospital care in an urban setting in Sabah, Malaysia. A prospective study of all patients younger than 12 years of age admitted with acute GE to Queen Elizabeth Hospital, Sabah, over a six-month period (October 2005 to March 2006) was conducted. During the study period, a total of 167 children with acute GE who had stool samples examined for RV were studied. RV accounted for 16% of all diarrhoeal cases, and 1.7% of all admissions to the children's wards during the study period. There was no difference in severity of GE between RV and non-RV groups. RV infection is a common cause of childhood GE requiring hospital care in Sabah.
Major congenital malformations occur in about 3% of newborn. Several studies have suggested that homozygosity for the C677T methylenetetrahydrofolate reductase (MTHFR) variant is a potential risk factor for neural tube defects (NTDs). It has been hypothesized that the maternal folic acid supplementation prevents NTDs by partially correcting reduced MTHFR activity associated with the variant form of the enzyme. This association has not been found in some ethnic groups. In this study, we attempted to assess the association between NTDs and MTHFR C677T in Malaysian Malay population. Results show that MTHFR 677TT genotype was absent in both patient and control groups.
To set the foundation for developing a centre for airway reconstruction, we performed a retrospective database review of patients operated at a tertiary-care university hospital. Over the past 3-year period from 2004 onwards, five paediatric cases of airway reconstruction procedures were performed. All cases had a two stages laryngotracheal reconstruction (TSLTR) for laryngotracheal stenosis (LTS). All patients were children below 15 years and the mean age was 9 years. Only one patient had a Grade IV Myer-Cotton stenosis, the rest all had Grade III stenosis. Three out of four of the Grade III stenosis patients were successfully decannulated within one year, the other one died of causes unrelated to LTS. The grade IV patient was still under followup and surgery was done only recently. This paper highlights the complexity of managing LTS in the paediatric age group and recommends the use of LTR with rib graft as a choice for the management of LTS.
BACKGROUND: Oral Langerhans cell histiocytosis is generally seen in children.
OBJECTIVE: To determine the clinicopathological features of oral LCH in Malaysian paediatric patients.
METHODS: A retrospective study was carried out to determine the clinicopathological features of Langerhans cell histiocytosis (LCH), Letterer-Siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma, and histiocytosis X occurring in the oral cavity in children, diagnosed histologically in the main oral histopathology laboratory in Malaysia from 1967 to 2007.
RESULT: There were 17 cases (eight girls and nine boys) with age ranging from 1 to 7 years. There were ten Malays, four Chinese, two Indians, and one of other ethnicity. Thirteen cases presented as gingival swellings with six of these cases accompanied with mobility of the teeth. Nine cases involved the mandible, two in the maxilla, and two cases in both the maxilla and mandible. The radiographic findings were mentioned only in nine cases with presence of bony erosion or destruction of the jaw bones. Four cases had punched-out radiolucencies of the skull. The patients also had other systemic signs and symptoms: skin lesions (n = 5), hepatosplenomegaly (n = 2), prolonged fever (n = 2), diabetes insipidus (n = 1), and exophthalmos (n = 1). Two cases were known cases of systemic LCH.
CONCLUSION: The histopathologic features of LCH are easily recognized; however, with the development of immunostaining, the use of CD1a helps in confirming the diagnosis.
From August 2006 to July 2007 a prospective study of out-of-pocket costs incurred by care-givers of children hospitalized for rotavirus gastroenteritis was conducted in a hospital in Malaysia. Data on caretaker out-of-pocket costs were collected from 260 children hospitalized with diarrhoea. A stool sample was collected from 198 of these children of which 46 (23%) were positive for rotavirus by latex agglutination assay. The mean (median; interquartile range) out-of-pocket cost incurred by the care-givers was US$194 (US$169; US$47-738), constituting 26% of average monthly income of the households surveyed. Major components of the cost were hospital expenses (45%) and productivity loss (37%). These findings will allow further assessment of the cost-effectiveness of any future rotavirus immunization program in Malaysia.
We reviewed cases of candidaemia at Universiti Kebangsaan Malaysia Medical Centre from 1st January 2005 to 30th June 2006. All blood cultures positive for Candida species or its teleomorphs within the study period were identified and antifungal susceptibility testing was performed. Out of 50 blood isolates, 20 (40%) were identified as Candida albicans, 16 (32%) C. tropicalis, five (10%) C. parapsilosis, three (6%) C. famata, two (4%) C. glabrata, two (4%) Pichia ohmeri, one (2%) C. krusei and one (2%) P. etchell/carsonii. Susceptibility to amphotericin B was 100%, fluconazole 90%, itraconazole 40%, ketoconazole 88%, 5-flucytosine 98% and voriconazole 98%.