A comparative pilot study was conducted to determine the difference in the reduction of total serum bilirubin in a group of infants who had phototherapy at home compared to an in-patient group on hospital phototherapy. Eighteen infants with unconjugated hyperbilirubinaemia who fitted the selection criteria were put under the mobile home unit (Bluelite Portable Light) placed in the home. A control group of 18 infants with the same matching characteristics had intense phototherapy in the hospital using a unit with top and bottom light sources. The infants were matched for race, starting total serum bilirubin level, birth weight (up to 250 grams) and age of baby at initiation of phototherapy (up to one-day difference). It was observed that the mean daily decrease in serum bilirubin concentration was significantly more in the home group as compared to the hospital group (t=2.95, df=17, P<0.05). The mean duration of treatment was significantly less for the home group as compared to the hospital group (t=2.84, df=17, P<0.05). None of the infants who had home phototherapy were re-hospitalized. Phototherapy related complications were mild and comparable in both groups. The result suggests that home phototherapy is safe and effective in bringing down the concentration of serum bilirubin for term babies with uncomplicated hyperbilirubinaemia.
We present two siblings from a consanguineous Malaysian family with multiple skeletal abnormalities, anterior segment anomalies of the eye and early lethality. These features are consistent with a syndrome first described by Al-Gazali and we provide further delineation of the syndrome.
Acquired subglottic stenosis is a compication or neonatal endotracheal intubation. Although it is rare, it contributes significantly to the morbidity and physical well being of post extubated neonates. A 20-day old neonate, ventilated for meconium aspiration syndrome and persistent fetal circulation, presented with marked stridor and respiratory embarrassment. A stenosed subglottic area was found on rigid bronchoscopy. Anterior cricoid split was performed to relieve the obstruction. He is asymptomatic post operatively.
RetCam is an excellent screening tool for the detection of retinopathy of prematurity (ROP). However, affordability is a barrier when adopting the use of RetCam in developing countries. We aimed to describe different stages of ROP using ultrasonographic B-scan and to evaluate the association between funduscopic examinations and ultrasonographic B-scan findings in premature neonates with ROP in Malaysia. A descriptive cross sectional study was conducted in 90 eyes of 47 premature neonates with different stages of ROP in three tertiary hospitals in Malaysia. Experienced ophthalmologists performed detailed funduscopic examinations using binocular indirect ophthalmoscopy (BIO). A masked examiner performed a 10 MHz ultrasonographic B-scan evaluation with 12 meridian position images within 48 hours of clinical diagnosis. Data from the clinical examination and ultrasonographic findings were collected and analysed. We recruited 37 eyes (41.1%) with stage 1 ROP, 29 eyes (32.3%) with stage 2, 18 eyes (20.0%) with stage 3, and 3 eyes (3.3%) with stages 4 and 5 based on the clinical assessment. Ultrasonography correctly identified 3 (8.1%) stage 1 eyes, 17 (58.6%) stage 2 eyes, 13 (72.2%) stage 3 eyes, and 3 each (100%) of the stage 4 and 5 eyes. There was a significant association between the funduscopic signs and the ultrasound findings for stage 2 ROP and above (Fisher's exact test, p <0.001). In conclusion, all stages of ROP were detected and described with a 10 MHz ultrasonic B-scan system. A significant association was observed between funduscopic signs and ultrasonographic findings in premature Malaysian neonates with stage 2 ROP and above.
The objectives of this prospective, observational study were to determine the current indications of requesting chest radiograph in sick infants in a neonatal intensive care unit (NICU) and the effect of a user-guided request form for chest radiographs of sick infants. During the three-month study period, a total of 423 chest radiographs were requested on 159 sick newborn infants in the intensive area of this NICU. A majority (55.6%) of these chest radiographs were performed to verify positions tips of either central catheters (27.4%) or endotracheal tubes (28.1%). The number of chest radiographs done during the period when the user-guided request forms were utilised was significantly lower (1.24 per patient) than before (1.37 per patient) or after (1.58 per patients) the period when these forms were in use (p=0.01). The rate of radiological abnormalities detected in radiographs requested to verify position of tips of endotracheal tubes were significantly greater during the period when user-guided forms were used than when they were not (p=0.01). A significantly higher proportion of changes in management were instituted when the user-guided forms were in use than during the period when they were not used (p=0.03). We conclude that a user-guided radiographic request form helps doctors in NICU to carry out their management more effectively.
A 1.7 kg infant with obstructed supracardiac total anomalous pulmonary venous drainage (TAPVD) presented with severe pulmonary hypertension secondary to vertical vein obstruction. The child, in addition, had a large omphalocele that was being managed conservatively. The combination of low weight, unoperated omphalocele, and severe pulmonary hypertension made corrective cardiac surgery very high-risk. Therefore, transcatheter stenting of the stenotic vertical vein, as a bridge to corrective surgery was carried out. The procedure was carried out through the right internal jugular vein (RIJ). The stenotic segment of the vertical vein was stented using a coronary stent. After procedure, the child was discharged well to the referred hospital for weight gain and spontaneous epithelialization of the omphalocele. Stenting of the vertical vein through the internal jugular vein can be considered in very small neonates as a bridge to repair obstructed supracardiac total anomalous venous drainage.
Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is the commonest cause of neonatal jaundice in Malaysia. Recently, OSMMR2000-D G6PD Assay Kit has been introduced to quantitate the level of G6PD activity in newborns delivered in Universiti Kebangsaan Malaysia Medical Centre (UKMMC). As duration of sample storage prior to analysis is one of the matters of concern, this study was conducted to identify the stability of G6PD enzyme during storage. A total of 188 cord blood samples from normal term newborns delivered at UKMMC were selected for this study. The cord bloods samples were collected in ethylene-diamine-tetra-acetic acid (EDTA) tubes and refrigerated at 2-8 °C. In addition, 32 out of 188 cord blood samples were spotted on chromatography paper, air-dried and stored at room temperature. G6PD enzyme activities were measured daily for 7 days using the OSMMR2000-D G6PD Assay Kit on both the EDTA blood and dried blood samples. The mean value for G6PD activity was compared between days of analysis using Student Paired T-Test. In this study, 172 out of 188 cord blood samples showed normal enzyme levels while 16 had levels corresponding to severe enzyme deficiency. The daily mean G6PD activity for EDTA blood samples of newborns with normal G6PD activity showed a significant drop on the fourth day of storage (p < 0.005) while for samples with severely deficient G6PD activity, significant drop was seen on third day of storage (p = 0.002). Analysis of dried cord blood showed a significant reduction in enzyme activity as early as the second day of storage (p = 0.001). It was also noted that mean G6PD activity for spotted blood samples were lower compared to those in EDTA tubes for all days (p = 0.001). Thus, EDTA blood samples stored at 2-8 °C appeared to have better stability in terms of their G6PD enzyme level as compared to dried blood samples on filter paper, giving a storage time of up to 3 days.
The initial clinical and hematologic presentation of infantile malignant osteopetrosis may be indistinguishable from that of juvenile myelomonocytic leukemia in infants. Timely radiographic imaging, however, allows straightforward delineation of these 2 severe diseases and facilitates immediate initiation of appropriate therapy.
A nonconsanguineous asymptomatic couple, were identified as carriers of factor VII (FVII) deficiency when two of their newborn children died of massive intracranial hemorrhage secondary to severe congenital FVII deficiency. Complete sequence analysis of the factor VII (F7) gene in this couple indicated that the mother was heterozygous for an A to G transition at position -2 of the exon 5 acceptor splice site, and the father was heterozygous for a G to T transversion at position +1 of the exon 6 donor splice site. This information allowed us to exclude a compound heterozygous deficiency state in a subsequent pregnancy using PCR/direct sequencing of the F7 gene using DNA obtained from chorionic villi at 10 weeks' gestation. Our experience with the family reported here further supports the conclusion that mutation-specific detection is reliable in the prenatal exclusion of severe bleeding disorders.
The incidence of West syndrome (WS) was determined by a search of reports of electroencephalograms (EEG) recorded in 1998 and 1999 in all public hospitals in Singapore. Amongst records of patients born in 1998, nine were found with EEG features of hypsarrhythmia or modified hypsarrhythmia with onset of seizures between January 1,1998 and December 31, 1999. The medical records of these patients were reviewed. The population of children born in 1998 was 43,664. In 1998 and 1999, 67% of all hospital admissions for patients 2 years or younger in Singapore were in public hospitals. The cumulative incidence of WS in Singapore corrected for the percentage of hospital admissions to public hospitals was 3.1/10,000 live births. The corrected cumulative incidences in Chinese, Malays and Indians were 2.7, 3.1 and 3.3 per 10,000, respectively. Three cases were idiopathic; three were due to congenital structural lesions of the brain; one each had periventricular leucomalacia, intracranial hemorrhage and severe intrauterine growth retardation. None of the patients were normal at follow up. The three patients with idiopathic WS had mild global developmental delay and the other six cases had cerebral palsy and severe mental retardation. With the best modern medical treatment, possibly only two of the nine cases of WS may have been prevented.
Interstitial lung disease (ILD) is very rare in children. In the majority of cases the aetiology is unknown. Very little is known about the clinical course of this condition in children. Prognosis may be influenced by sex, age of onset of symptoms, radiographic features, presence of right ventricular hypertrophy and histopathology. We report our experience in managing four children with interstitial lung disease. All these children presented in early infancy with cough, respiratory distress, cyanosis and failure to thrive. Three of these children had finger clubbing and right ventricular hypertrophy. All patients received oral steroids. Chloroquine was added in two patients who showed no response. A trial of oral cyclophosphamide was started in one patient who failed with both drugs. One child is oxygen independent while another is on home oxygen therapy. The other two patients eventually died.
Respiratory syncytial virus (RSV) is the most important cause of lower respiratory tract infection (LRTI) in young children. We determined if there was a seasonal variation in Malaysia in the incidence of RSV infection in young children admitted with LRTI, and possible associations of RSV infection with local meteorological parameters. A total of 5,691 children, aged less than 24 months and hospitalized with LRTI (i.e., bronchiolitis and pneumonia) between 1982-1997, were included in this study. Nasopharyngeal samples were collected and examined for RSV by immunofluorescence, viral culture, or both. Seasonal variations were determined by analyzing the monthly RSV-positive isolation rate via time series analysis. Possible correlations with local meteorological parameters were also evaluated.RSV was isolated in 1,047 (18.4%) children. Seasonal variations in RSV infection rate were evident and peaked during the months of November, December, and January (test statistics [T] = 53.7, P < 0.001). This seasonal variation was evident for both bronchiolitis and pneumonia categories (T = 42.8 and 56.9, respectively, P < 0.001). The rate of RSV infection appeared to correlate with the monthly number of rain days (r = 0.26, P < 0.01), and inversely with the monthly mean temperature (r = -0.38, P < 0.001). In the tropics, seasonal variations in the incidence of RSV infection are evident, with an annual peak in November, December, and January. This information provides a guide for healthcare provisions and implementation of RSV prevention.
The risk factors and modes of death following acute diarrhoeal illness in children admitted to University Hospital, Kuala Lumpur between 1982 and 1997 were studied retrospectively. Among 4,689 cases of acute gastroenteritis admitted, ten deaths were noted. The case mortality rate was 2.1/1000 admissions. All deaths were infants below one year, with eight females and two males. Acute renal failure and acute pulmonary oedema were common preceding events. Female sex, infants less than twelve months, the presence of hyper or hyponatraemia and moderate to severe dehydration on admission were risk factors for deaths.
A survey of domestic childhood accidental injuries was conducted at a rural general practice in Arau, Perlis. Data was collected from parents or other caregivers of 171 children, aged 12 years and below, using a pretested questionnaire. Male children between the ages of 6 and 12 years were the most common group affected, with a male to female ratio of 1.7:1. The three most common accidents were injuries from falls (28%), cuts, lacerations, bruises and puncture wounds not resulting from falls (26%), and thermal injuries (22%). The most commonly affected parts of the body were the limbs. Most injuries to children between ages 4 to 12 years occurred in the house compounds, while those to children below 4 years occurred in the kitchen and other locations within the house. Major contributing factors to the injuries were the existence of unsafe home environments, the risk taking activities of the children, the presence of hazardous products in the household and unrealistic parental attitudes to injury prevention.
The application of a continuous distending pressure (CDP) has been shown to have some benefits in the treatment of pre-term infants with respiratory distress syndrome (RDS). CDP has the potential to reduce lung damage, particularly if applied early before atelectasis has occurred. Early application of CDP may better conserve an infant's own surfactant stores and consequently be more effective than CDP applied later in the course of RDS.
A study on infant feeding practices was conducted during the implementation of the Baby Friendly Hospital Initiative (BFHI) in a district hospital. The aim was to identify which population subgroups had lower breastfeeding rates at 4 months and the effect of attendance of antenatal breastfeeding education on breastfeeding practices. All infants delivered in May 1996 were followed-up. 204 respondents were analyzed. This study demonstrated a higher exclusive and any breastfeeding rates at 4 months than some other studies. (48% and 76% respectively). It was found that the Malays were more likely to be breastfeeding exclusively at 4 months (72%) than the Indians (32%) and the Chinese (4%). (P < 0.01). There were more non-working mothers breastfeeding exclusively at 4 months than working mothers. (60% versus 26%) P < 0.01. Antenatal breastfeeding education in the form that was given appeared to improve breastfeeding rates at 4 months. Future efforts to promote breastfeeding should target the Chinese mothers and the working mothers.
An 18-month analysis of 52 percutaneously placed central venous catheters in 48 critically ill children was done. Success rate were 91.7% (33/36) and 93.8% (15/16) for femoral and non-femoral catheters respectively. Presence of hypotension (48.1%) and significant coagulopathy (26.9%) did not affect the success rate significantly. Minor bleeding and venous congestion was seen in 5.5% (2/36) of patients with femoral catheters. Infections were found in 2.7% (1/36) of femoral and 6.6% (1/15) of non-femoral catheters. The low incidence of complications and the relative ease of insertion makes the femoral route the preferred site for trainee medical officers in critically ill children when central access is indicated.
Summary: Home oxygen therapy programme is new in Malaysia. This programme enables children with respiratory insufficiency to be discharged home early.
Materials and Methods: Long term oxygen therapy was initiated using an oxygen concentrator in patients who i) remained hypoxic while breathing room air, ii) experienced desaturations of more than 20% during sleep as seen in patients with severe laryngomalacia and obstructive sleep apnoea syndrome and iii) had pulmonary hypertension with or without polycythemia. The median with first and third quartile values are presented for the quantitative variables.
Results: A total of 71 patients mainly children with bronchopulmonary dysplasia (BPD) (32) and bronchiolitis obliterans (12) were discharged home on this programme. The median age at which home oxygen was initiated in children with BPD was 5.0 (Q1:2 Q3:8) months. The median total duration of oxygen requirement for BPD was 8.0 (Q1:5, Q3:12) months. The median duration of home oxygen dependency was 3.5 (Q1:3, Q3:6) months. However children with bronchiolitis obliterans required longer duration of oxygen therapy compared to children with BPD i.e. median duration of 28 months (Q1:14.5 Q3:66). In other respiratory conditions the mean duration of supplemental oxygen varies some of which may be life long.
Conclusions: This paper has shown the importance of home oxygen program in children with respiratory disorders. It has significantly shortened hospital stay and thus saves hospital costs and prevents prolonged separation from the family.