A prospective study was carried out on 26,176 Malaysian neonates born in the Maternity Hospital, Kuala Lumpur over a 12-month period to determine the incidence and associated risk factors of brachial plexus injuries. This condition was found in 42/26,176 neonates (1.6 per 1000 livebirths). Multiple logistic regression analysis of affected and control neonates from a nested case-control study showed that increasing birth weights and breech deliveries were the significant risk factors. Our study suggests that to reduce the occurrence of this condition, there is a need for: (i) better assessment of fetal size and maternal pelvimetry to enable earlier diagnosis of cephalo-pelvic disproportion, and (ii) review of the indications and techniques of breech delivery.
The early identification of asphyxiated infants at high risk of adverse outcomes and the early selection of those who might benefit from neuroprotective therapies are required. A prospective observational study was conducted to determine if there were any early clinical, neuroimaging or neurophysiological parameters that might predict the outcome in term newborns with asphyxia.
Chylothorax is a rarely recognised post-operative complication following repair of congenital diaphragmatic hernia. We report here a newborn infant with this condition which resolved with percutaneous chest drainage, total parenteral nutrition and enteral feeding of a formula high in medium-chain triglycerides.
The aim of this study was to compare the response and survival rates of term infants with persistent pulmonary hypertension of the newborn (PPHN) on high frequency oscillatory ventilation (HFOV) treated with either inhaled nitric oxide (iNO) or intravenous magnesium sulphate (MgSO4).
This study aimed to determine the rates of non-adherence to standard steps of medication administration and medication administration errors committed by registered nurses in a neonatal intensive care unit before and after intervention.
INTRODUCTION:
This study aimed to compare the detection rates of glucose-6-phosphate dehydrogenase (G6PD) deficiency in neonates by fluorescent spot test (FST), enzyme assay and molecular methods, and to identify which method was a significant predictor of severe hyperbilirubinaemia.
METHODS:
74 term infants of Chinese descent admitted with severe hyperbilirubinaemia (total serum bilirubin equal or greater than 300 micromol/L) and 125 healthy term infants born in the hospital without severe hyperbilirubinaemia were recruited into the study. Specimens of blood were collected from each infant for FST, G6PD enzyme assay and TaqMan minor groove binder single nucleotide polymorphism genotyping assay.
RESULTS:
26 (13.1 percent) infants were diagnosed to have G6PD deficiency by FST. They had significantly lower median enzyme levels (0.8 IU/g Hb, interquartile range [IQR] 0.4-4.3) than those diagnosed to be normal (12.0 IU/g Hb, IQR 10.3-15.8) (p-value is less than 0.0001). Based on the enzyme assay, 39 (19.6 percent) infants had G6PD deficiency at an enzyme cut-off level of less than 8.5 IU/g Hb. G6PD mutation was detected in 27 (13.6 percent) infants. Logistic regression analysis showed that the only significant predictors of severe hyperbilirubinaemia were G6PD deficiency based on a cut-off level of less than 8.5 IU/g Hb (adjusted odds ratio [OR] 5.3, 95 percent confidence interval [CI] 2.4-11.4; p-value is less than 0.0001) and exclusive breast-feeding (adjusted OR 11.4, 95 percent CI 3.1-42.4; p-value is less than 0.0001). The gender and birth weight of infants, FST results, G6PD mutation and the actual G6PD enzyme levels were not significant predictors.
CONCLUSION:
A G6PD enzyme level of less than 8.5 IU/g Hb is a significant predictor of severe hyperbilirubinaemia
Genes on the Y chromosome are essential for normal sex determination and sex differentiation of male genitalia. However, genes on the X chromosome and other autosomes have been shown to be anti-testes and have a detrimental effect on this process. Addition of X chromosomes to the 46,XY karyotype results in seminiferous tubules dysgenesis, hypogonadism and malformed genitalia. We report a term male newborn with 49,XXXXY syndrome presenting with ambiguous genitalia, multiple extra-gonadal anomalies, facial dysmorphism, and radioulnar synostosis.
Introduction: The aim of this study was to determine predictors of failed closure of patent ductus arteriosus (PDA) following a single course of indomethacin in symptomatic preterm infants.
Methods: This prospective observational study was carried out on 60 preterm infants weighing less than 1,750 g with symptomatic PDA confirmed by echocardiography. At a median age of 7.0 days (interquartile range 4.0), they were given indomethacin of 0.1 mg/kg/day intravenously daily for six days. Closure of PDA was reassessed by echocardiography upon completion of therapy.
Results: The PDA of 40 percent (n=24) of these infants remained patent. Forward logistic regression analysis showed that the only significant predictors of failed PDA closure in these infants were: PDA size (adjusted odds-ratio [OR] is 7.0; 95 percent confidence interval [CI] of OR is 2.0, 24.8; p-value is 0.002), birth weight (adjusted OR is 0.996; 95 percent CI of OR is 0.993, 1.000; p-value is 0.03) and platelet count (adjusted OR is 0.987; 95 percent CI is 0.975, 1.000; p-value is 0.045). Gestational age, maternal age and left atrium/aorta ratios were not significant predictors.
Conclusion: Larger PDA, lower birth weight and lower platelet count were significant predictors of high failure in indomethacin therapy given late at one week of life.
A 2-year study was carried out in the Maternity Hospital, Kuala Lumpur to determine the neonatal mortality rates. This Hospital functions both as the local service centre as well as the national referral centre in Malaysia. Its neonatal services, however, were equipped and manned at those below Level III perinatal centre. During the study period 52, 877 livebirths took place in the Hospital. In 1987 and 1988 respectively, the low birthweight (less than 2500 gm) rates were: 112.8 and 101.9 per 1000 livebirths, very low birthweight (less than 1500 gm) rates: 11.1 and 8.8 per 1000 livebirths, neonatal mortality rates: 12.5 and 10.7 per 1000 livebirths and neonatal mortality risk ratio: 1.15 and 1.27. There was significant difference in mortality rates among the Malay, Chinese and Indian babies born in this hospital: the Indians had the highest and the Chinese the lowest rates. Babies delivered by breech or lower segment Caesarean section (LSCS) also had significantly higher mortality than those delivered by other modes of delivery. Low birthweight neonates constituted less than 45% of the total special care nursery admission but contributed to more than 70% of the total neonatal deaths. The common causes of neonatal deaths were problems of prematurity, infection, asphyxia and congenital malformations. Preterm and low birthweight neonates died primarily from problems of prematurity or infection. Term and larger neonates died mainly from asphyxia. More than 75% of the neonatal deaths occurred before 7 days of life. Improvement of antenatal care in the community and upgrading of perinatal services in this Hospital could help to lower the morbidity and mortality due to preventable causes.
To determine the risk factors for rectal colonization by extended-spectrum beta-lactamase (ESBL) Klebsiella sp. in 368 newborns admitted consecutively to a neonatal intensive care unit over six months, rectal swabs were cultured on admission and weekly until discharge. Eighty infants (21.7%) had ESBL Klebsiella sp. cultured from their rectal swabs. Eighty controls were selected at random from infants with negative cultures admitted within the 14-day period prior to the detection of ESBL Klebsiella sp. in the cases. Cases had significantly lower birth weight, gestational age, earlier age of admission, longer hospital stay, and higher proportions of congenital malformations, early-onset pneumonia and respiratory distress syndrome compared with controls. Significantly more cases received mechanical ventilation, nasal continuous positive airway pressure support, total parenteral nutrition, umbilical vascular catheterization, arterial line insertion, urinary bladder catheterization, and prior treatment with antibiotics. However, stepwise logistic regression analysis showed that only two independent risk factors were significantly associated with ESBL rectal colonization: duration of hospital stay [adjusted odds ratio (OR): 1.3; 95% confidence intervals (CI): 1.2, 1.4; P<0.0001) and early-onset pneumonia (adjusted OR: 8.3; 95% CI: 1.6, 43.4; P=0.01).
The burden of non-communicable diseases is increasing in Malaysia. Insufficient Physical Activity, which is an important risk factor for non-communicable diseases, is less researched in Malaysia. We aimed to assess the level of physical activity and identify its correlates. An online survey was carried out during October, 2011 in the University Tunku Abdul Rahman by the opinion poll research committee. Young adults answered the Short International Physical Activity Questionnaire and a questionnaire about factors according to a socio-ecological model which was adapted from published studies. Metabolic equivalent (MET)-hours and MET-minutes were calculated. Physical activity was classified as sufficient when MET-minutes were > 840. The mean age of the 474 participants was 22.4 years (S.D. = 4.7), and 253 (53.4%) were females. Their mean and median of MET-hours of PA done during the previous seven days were 31.36 (S.D., 52.19) and 14.7 (IQR, 5.77-32.07), respectively. Physical activity done was sufficient among 242 (51.1%) participants. Using univariate analysis, being male, good self-rated health, positive intention, self-efficacy, perceived benefits, social support, and availability of facilities were associated with sufficient physical activity. Using multivariate analysis sufficient physical activity was associated with participants' intention (OR 0.75, 95% CIs 0.64, 0.88), self-efficacy (OR 0.91, 95% CIs 0.85, 0.97) and facility availability (OR 0.81, 95% CIs 0.73, 0.91). The proportion of participants with sufficient physical activity was low. Positive intention and self-efficacy associated with sufficient physical activity should be supported by availability of facilities and a safely-built environment. A nationwide survey about physical and associated socialecological factors is needed to design rational health promotion strategies.
This study aimed to determine the gaps of knowledge and practices of care of neonatal jaundice among Malaysian mothers. It was a cross sectional study of 400 mothers who attended the obstetric clinics or were admitted to the obstetric wards of a general hospital. They were surveyed with a structured set of questionnaire. The results showed that a majority (93.8%) of them knew about neonatal jaundice, and 71.7% knew that jaundice lasting more than 2 weeks was abnormal. However, only 34.3% of them were aware that jaundice appearing during the first 36 hours of life was abnormal. Less than 20% knew about glucose-6-phosphate dehydrogenase deficiency and that fetal-maternal blood group differences could cause jaundice. Although 71.7% and 69.7%, respectively, of the mothers knew that severe jaundice could cause death and brain damage, only 38.4% of them were aware that severe jaundice could result in hearing impairment. A very low proportion (27.1%) of them was aware that putting jaundiced infants under the direct sun could result in dehydration and worsening of jaundice. Out of a maximum score of 15, the mean maternal knowledge score was 7.4 (95% confidence intervals: 7.1, 7.7). Majority (83.1%) of the multiparous mothers with a past history of having children developing neonatal jaundice (n = 154) practiced placing their infants under the direct sun. This study revealed that there was a wide knowledge gap among Malaysian mothers on care of neonatal jaundice. Placing infants under the direct sun was still a common practice.
Study site: Obstetric clinic, Hospital Tuanku Jaafar, Seremban, Negeri Sembilan, Malaysia
A cross-sectional study was undertaken to determine and compare the refractive status of premature children without retinopathy of prematurity (ROP) and full term children below the age of three years.
Transient abnormal myelopoeisis (TAM) is a haematological phenomenon commonly seen in newborns with Down syndrome. Although the majority show spontaneous resolution, this condition should not be dismissed too readily as there have been associated fatalities. Furthermore, even for those who do show spontaneous resolution, a significant percentage will develop acute megakaryoblastic leukaemia within the next few years of life. We report a series of four patients with TAM who presented with hepatosplenomegaly and leucocytosis detected on preliminary investigations.
Recurrent spontaneous abortion, defined as three consecutive abortions, occurs in approximately 1% to 2% of couples. Although the cause is unknown in up to 50% of cases, about 5% of these couples are found to be a balanced translocation carrier. We report a case in which the mother was identified to be a translocation carrier following the birth of a baby with multiple congenital abnormalities.
Factor VII deficiency is a rare congenital blood disorder. Its clinical features are rather variable and ranges from epistaxis to massive intracranial haemorrhage. Treatment involves replacement therapy, which constitutes use of fresh frozen plasma, prothrombin complex concentrates or recombinant activated factor VII. Although it is a rare entity, one still needs to consider it as a probable diagnosis in a newborn with coagulopathy. We report here a case of Factor VII deficiency in a newborn who presented with subdural haemorrhage at day 4 of life.
Ureaplasma urealyticum was isolated from the endotracheal aspirates of 39 (21.4%) of 182 neonates with respiratory distress requiring ventilatory support. Mycoplasma hominis was isolated from one (0.5%) neonate. Bacterial cultures were negative in 123 (67.6%) neonates. Antibiotic susceptibility test carried out on ten isolates of U. urealyticum showed that all the organisms were sensitive to erythromycin but resistant to lincomycin and sulfamethoxazole trimethoprim. All, except one, U. urealyticum were sensitive to tetracycline and minocycline. Two isolates were resistant to ciprofloxacin. This study showed that U.urealyticum was a common organism isolated from the endotracheal aspirates of neonates with respiratory distress.
We screened 38 G6PD-deficient male Chinese neonates for known G6PD mutations using established PCR-based techniques. We found 50.0% (19 of 38) were mutation 1376G>T, 34.2% (13 of 38) were mutation 1388G>A, 5.2% (2 of 38 ) were mutation 95A>G and 2.2% (1 of 38) was mutation 1024C>T. In 7% (3 of 38) of the cases the mutations remained uncharacterised. Sixty three percent (24 of 38) of the G6PD deficient neonates had neonatal jaundice with 28.9 % (11 of 38) developing moderate to severe hyperbilirubinemia. The group of neonates with 1388 mutation showed the highest incidence of moderate to severe hyperbilirubinemia requiring phototherapy and/or exchange transfusion respectively. Majority (70%) of the G6PD deficient neonates showed severe enzyme deficiency. However, there was no meaningful association between the level of enzyme activity and the severity of neonatal jaundice. In summary, four mutations account for more than 90% of the G6PD deficiency cases among the Chinese in Malaysia and the pattern of distribution of the molecular variants is similar to those found among the Chinese in Taiwan and southern mainland China. Our findings also suggest the possible association of nt 1388 mutation with severe neonatal jaundice.
We performed DNA analysis using cord blood samples on 86 male Malay neonates diagnosed as G6PD deficiency in the National University of Malaysia Hospital by a combination of rapid PCR-based techniques, single-stranded conformation polymorphism analysis (SSCP) and DNA sequencing. We found 37.2% were 871G>A (G6PD Viangchan), 26.7% were nt 563 C>T (G6PD Mediterranean) and 15.1% were 487G>A (G6PD Mahidol) followed by 4.7% 1376G>T (G6PD Canton), 3.5% 383T>C (G6PD Vanua Lava), 3.5% 592C>T (G6PD Coimbra), 2.3% 1388G>A (G6PD Kaiping), 2.3% 1360C>T (G6PD Union), 2.3% 1003G>A (G6PD Chatham), 1.2% 131C>G (G6PD Orissa) and 1.2% 1361G>A (G6PD Andalus). Seventy-one (82.6%) of the 86 G6PD-deficient neonates had neonatal jaundice. Fifty seven (80%) of the 71 neonates with jaundice required phototherapy with only one neonate progressing to severe hyperbilirubinemia (serum bilirubin >340 micromol/l) requiring exchange transfusion. There was no significant difference in the incidence of neonatal jaundice, mean serum bilirubin level, mean age for peak serum bilirubin, percentage of babies requiring phototherapy and mean number of days of phototherapy between the three common variants. In conclusion, the molecular defects of Malay G6PD deficiency is heterogeneous and G6PD Viangchan, Mahidol and Mediterranean account for at least 80% of the cases. Our findings support the observation that G6PD Viangchan and Mahidol are common Southeast Asian variants. Their presence in the Malays suggests a common ancestral origin with the Cambodians, Laotians and Thais. Our findings together with other preliminary data on the presence of the Mediterranean variant in this region provide evidence of strong Arab influence in the Malay Archipelago.
Neonatal screening for G6PD deficiency has long been established in many countries. The aim of the study was to determine whether the routine semiquantitative fluorescent spot test could detect all cases of G6PD deficiency, including those cases with partial deficiency (residual red cell G6PD activity between 20-60% of normal). We compared the results of G6PD screening by the semiquantitative fluorescent spot test and quantitative G6PD activity assay on a group of 976 neonates and 67 known female heterozygotes. The values for mean G6PD activity of G6PD-normal neonates and 293 healthy adult females were determined. There was no significant difference in the mean normal G6PD activity between the two racial groups in the neonates (669 Malays, 307 Chinese) and in the 293 healthy adult females (150 Malays, 143 Chinese) group. The values for the upper limits of total deficiency (20% of normal residual activity) for neonates and adult females were 2.92 U/gHb and 1.54 U/gHb, respectively. The upper limits of partial deficiency (60% of normal residual activity) were 8.7 U/gHb and 4.6 U/gHb respectively. The prevalence of G6PD deficiency among the male neonates was 5.1% (26) by both the fluorescent spot test and the enzyme assay method. The G6PD activity levels of all 26 cases of G6PD-deficient male neonates were < 20% normal (severe enzyme deficiency). In the female neonate group, the frequency of G6PD deficiency was 1.3% (6 of 472) by the fluorescent spot test and 9.35% (44 of 472) by enzyme assay. The 6 cases diagnosed as deficient by the fluorescent spot test showed severe enzyme deficiency (< 2.92 U/gHb). The remaining 38 female neonates had partial enzyme deficiency and all were misdiagnosed as normal by the fluorescent spot test. In the female heterozygote group, G6PD deficiency was diagnosed in 53% (35 of 67) by enzyme assay and in 7.5% (4 of 67) of cases by the fluorescent spot test. The 4 cases detected by fluorescent spot test had severe enzyme deficiency (<1.6 U/gHb). The remaining 31 (46.3%) cases, diagnosed as normal by fluorescent spot test, showed partial G6PD deficiency. In conclusion, we found that the semiquantitative fluorescent spot test could only diagnose cases of total G6PD deficiency and misclassified the partially-deficient cases as normal. In this study, the overall prevalence of G6PD deficiency was 3.28% by the semiquantitative fluorescent spot test and 7.17% by enzyme assay. This means that 3.9% of G6PD-deficient neonates were missed by the routine fluorescent spot test and they were found to be exclusively females. This study demonstrates a need to use a method that can correctly classify female heterozygotes with partial G6PD deficiency. The clinical implication is that these individuals may be at risk of the hemolytic complication of G6PD deficiency.