Newborns were examined for the presence of slow-moving haemoglobin components, tentatively designated X components and previously found in a group of Hb H disease in which invariably one of the parents of each patient had the same slow-moving Hb X components also. Structural studies showed that the abnormal haemoglobin in Chinese was identical with Hb Constant Spring, an c-chain variant. Newborns with Hb Bart’s and slow-moving X components invariably had one parent with the X components also. When the child grew older Hb Bart’s disappeared while the Hb X components remained in the blood. The homozygous state for the X components was found in a Malay boy through his newborn brother who had the X components in addition to Hb Bart’s and had both parents with the X components. One other Malay baby had the X components and Hb A2 Indonesia inherited from the parents. The present study of newborns also showed that Hb Bart’s can accompany different abnormalities of haemoglobin production, involving alpha-chains, beta-chains as well as gamm-chains. Its presence in cord blood is, therefore, not specific for alpha-thalassaemia
Key Words: Haemoglobinopathies; Hb Bart’s; Slow-moving Hb X; Thalassaemia
Objectives: The objectives of this study were to ascertain the prevalence of Indonesians obstetrics immigrant and to assess the fetal maternal outcome. Methodology: A prospective cohort study design was used to analyse 54 consecutive Indonesians obstetrics immigrant compared to 56 Malay women. Chi square and student t test were used where appropriate, p < 0.05 was considered to be of statistical significance. Results: There was a reducing trend in the incidence admission of Indonesians from 10.5 in 1999 to 6.5 % in 2002. The maternal mortality ratio showed an increasing trend from 1999 (40.0/100000) to 2001 (162.9/100000) but decreased to 5.8/100000 in 2002. The majority was between 20-40 years old, multiparous and booked, which was similar to the Malay population. Most Malays were in occupational class 1 to 3 and the husband has secondary and tertiary education compared to the Indonesians who were in class 4 and 5 and the husband has primary and no formal education. Significantly more immigrants were housewives compared to Malays who were working women. There was no significant difference in the antenatal complications. There were no significant difference in terms of delivery and most delivered vaginally. The perinatal outcome in terms of gestation, birth weight, Apgar score and admission to neonatal ICU were similar in both populations and there was no perinatal mortality. In conclusion, the incidence admission of Indonesian immigrant was on the decreasing trend. The outcome of these patients managed in MHKL was similar to the Malay population. Further studies with enrollment of a larger number of patients should be carried out to ascertain the significance of these findings.
Urinary tract infections frequently affect pregnant mothers. This problem causes significant morbidity and healthcare expenditure. Three common clinical manifestations of UTIs in pregnancy are: asymptomatic bacteriuria, acute cystitis and acute pyelonephritis. Escherichia coli remains the most frequent organism isolated in UTIs. All pregnant mothers should be screened for UTIs in pregnancy and antibiotics should be commenced without delay. Urine culture and sensitivity is the gold standard in diagnosing UTIs. Without treatment, asymptomatic bacteriuria in pregnancy is associated with preterm delivery, intrauterine growth retardation, low birth weight, maternal hypertension, pre-eclampsia and anaemia. Acute pyelonephritis can lead to maternal sepsis. Recurrent UTIs in pregnancy require prophylactic antibiotic treatment.
Two active interventions were carried out in order to encourage breast feeding of low birth weight (LBW) neonates who were admitted to the neonatal intensive care unit (NICU), Universiti Sains Malaysia. These were the establishment of (a) an effective transport system for conveying expressed breast milk (EBM) from the motherk home to the neonatal intensive care unit and (b) providing rooming-infacilities in the neonatal ward. The EBM was transported by a male health assistant, by the parents themselves or by the local bus company. A total of 473 babies were admitted from April 1994 to September 1995. Thirty-three babies weighed between 540-1000g and 440 were between 1001-2000g. A total of 226 (47.8%) babies were given mixed formula (less than 20% of daily needs) and EBM feeds (Group A) and 247 (52.2%) were given only formula feeds (Group B). A total of 79 babies died of which 5 were in Group A and 74 were in group B. (P value is 0.00). In Group A, 45 babies had clinical sepsis, 9 of whom were blood culture positive. Four of these babies died. In group B, out of 108 babies with clinical sepsis 36 were blood culture positive. 29 died because of the sepsis This study shows that by providing an effective transport system for breast milk expressed at home and the availability of roomingfacilities in the neonatal ward a significant number of LBW babies admitted to the NICU continue to receive their mothers' milk. The incidence of nosocomial sepsis and the over all mortality due to sepsis was significantly higher in those on full formulafeeding compared to those given mixed formula and expressed breast milk.
Nesidioblastosis is a rare metabolic disease characterised by inappropriate insulin secretion often associated with life-threatening hypoglycaemia. While severe cases present in the newborn period, patients have been described later in infancy. Familial cases suggest an autosomal recessive trait, and recently mutations in the sulphonlurea receptor gene, possibly a regulator of insulin secretion, have been identified and associated with disease expression. We report a twin boy who developed normally until the age of six months when he was noted to regress. The boy is the older twin born to non-consanguinous parents. He presented to a hospital first at the age of 13 months with fever and generalised seizures. Low blood glucose was noted, but he recovered easily and was able to maintain euglycaemia during a 48-hour period of observation. Microcephaly and developmental delay were documented and anticonvulsant therapy was started. At 18 months, low blood glucose with high C-peptide was documented during reevaluation. Follow-ing a short trial of subcutaneous long-acting somatostatin analogue, the child was subjected to near-total pancreatectomy. The histology revealed findings consistent with nesidioblastosis. The child's condition improved but he remained significantly delayed This case emphasises the importance of recognising and treating hypoglycaemia early to avoid irreversible brain damage. It is interesting to note that the twin brother has always been well and is developmentally normal. Further studies to identify the inheritance pattern in the family would be of great interest.
Vitamin B12 deficiency is a well-known cause of megaloblastic anaemia and pancytopenia. However, the incidence in pregnancy is rarely reported. We present a case of a 32-year old multigravid woman who was diagnosed with megaloblastic anaemia since 22 weeks gestation and progressed to develop severe pancytopenia at 30 weeks gestation. She was also diagnosed with vitamin B12 deficiency related to dietary and sociocultural habits. Folate and iron levels were normal throughout pregnancy. Treatment with parenteral cyano-cobalamin resulted in sustained improvement of haematological parameters. The pregnancy was carried to term and the baby was born weighing 2,050gm but otherwise well at birth and had normal developmental milestones thereafter. This case illustrates the clinical presentation of maternal vitamin B12 deficiency and demonstrates the importance of detecting and treating maternal vitamin B12 deficiency during pregnancy in at-risk patients. Failure to diagnose and institute treatment carries significant risks to both mother and child. Oral vitamin B12 supplementation should be considered for patients who are strict vegetarians or consume very little animal products.
A 46 day old female Chinese infant was referred for fail-ure to thrive, jaundice, hepatomegaly and bilateral cataracts. She had vomiting,blood stained stools and severe unconjugated hyperbilirubinaemia soon after birth. The jaundice persisted. At one month of age, pale stools, firm hepatomegaly and bilateral cataracts were noted. Radionuclide hepatobiliary scintigraphy per-formed at another hospital excluded biliary atresia. Investigations showed cholestasis but a negative sero-logical screening for congenital infections. A presump-tive diagnosis of galactosaemia was made and the infant was started on lactose free formula. A deficient red blood cell galactose- 1 -phosphate uridyltransferase (GALT) activity was demonstrated later. Review eight months after the initial diagnosis showed a thriving infant with no jaundice, but persisting cataracts and firm enlarged liver. A high index of clinical suspicion, labo-ratory confirmation of a deficient GALT activity and prompt withdrawal of lactose from diet are necessary to avoid any delay in diagnosis and management of this condition.
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.
The aim of the study was to determine the effect of pro-phylactic low dose dopamine infusion on renal function in ventilated premature newborns with respiratory dis-tress syndrome (RDS). A prospective, randomised con-trolled trial was conducted, using low dose dopamine [2.5μg/kg/min] in the treatment of preterm babies with gestational age 28-36 weeks requiring mechanical ventilation for RDS within six hours of age. Thirty-six babies were enrolled and 19 babies were randomly assigned to the treatment groups. The renal function after 72 hours for the treatment and control groups respectively were: urine output (ml/kg/hour) 3.3±0.4 and 3.0±0.3 [p=0.55], urine specific gravity 1006±0.6 and 1006±1.0 [p=0.68], fractional excretion of sodium 4.1±0.8 and 2.6±0.4 [p=0.10], fractional excretion of potassium 37.44 ± 5.6 and 16.49 ± 2.2 [p=0.001], glomerular filtration rate (ml/day/1.72m2) 16±2.6 and 25.6±4.5 [p=0.06]. There were no significant differ-ences in the frequency of hypotension, oliguria and sep-sis between the two groups. There were seven deaths (36.8%) in the treatment group (six due to sepsis and one due to prematurity) and two deaths (11.8%) in the control group (both due to sepsis) (p = 0.13). In con-clusion prophylactic low-dose dopamine infusion did not improve the renal function in ventilated premature babies with respiratory distress syndrome. The results of this study do not support the routine use of prophylac-tic low-dose dopamine in ventilated preterm babies with respiratory distress syndrome.
The objective of this study was to examine the effect of surfactant replacement therapy on hospital resource uti-lization in a well defined cohort of preterm infants with respiratory distress syndrome (RDS). Thirty preterm infants 800g with RDS requiring mechanical ventila-tion with Fi02 of 0.4 given modified bovine surfactant (Survanta) were compared with an equal number of his-torical controls. The total cost of neonatal care was cal-culated in a detailed survey covering all aspects of resource use. Surfactant-treated infants had an improved survival rate (90.0% vs 66.7%, p=0.03) and a trend towards shorter ventilator days (11.8 vs 19.0 days, p=0.17). There were no significant differences in the number of laboratory and radiological investiga-tions, use of disposable items, equipment, medications and other therapies. The total hospital cost per livebirth for surfactant-treated and control infants were R/V120,281 and R1V121,785 respectively. Personnel salaries represented the largest sector of resource uti-lization. When analysed by birthweight categories, the cost per livebirth for surfactant-treated and control infants in the 800-999g category were RM37,315 and RM14,760 respectively. As for the surfactant-treated and control infants in the 1000-1499g category, the cost per livebirth were RM21,426 and RM32,327 respectively. We concluded that surfactant replacement therapy did not increase overall hospital resource uti-lization and may decrease the cost for infants weighing 1000g.
ANTENATAL CORTICOSTEROID THERAPY. Benefits. In 1969, the first study was published which showed that prematurely delivered lambs exposed prenatally to corticosteroids survived longer than placebo-treated control animals.' A randomised clinical trial (RCT) followed which demonstrated that antenatal corticosteroid therapy significantly reduced the incidence of respiratory distress syndrome (RDS) in infants born before 2 weeks gestation and reduced mortality in those born before 37 weeks.2 A meta-analysis has been published on 12 RCTs involving over 3000 women in preterm labour, using primarily 24mg of betamethasone or dexamethasone given in 2-6 divided doses over a 48-hour period.' It showed that antenatal corticosteroid therapy is associated with a significant reduction in the risk of RDS (a) if the infant is born > 24 hours or < 7 days of the treatment, (b) in both male and female infants and (c) even in infants < 31 weeks gestation. It also significantly reduces mortality rate and morbidity such as intraventricular haemorrhage (IVH) and necrotising enterocolitis (NEC), shortens the duration of hospitalisation and reduces treatment costs. The improvement in survival rate in infants born
The objectives of this study were: (1) to detect group B streptococcus (GBS) carriers among antenatal patients during pregnancy ( 35 to 37 weeks) in the University Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia, and (2) to give antibiotic prophylaxis during the intrapartum period for the GBS carrier patients. The aim is to prevent early onset of GBS infection in newborn. This pilot study was carried out from 1st July 2005 to 31st August 2005. It is a prospective study involving 56 pregnant women who were seen at the antenatal clinic in the UMMC between 35 to 37 weeks gestation. High vaginal swab and low vaginal together with endoanal swab were taken for culture and sensitivity. The antibiotic prophylaxis was started early in the intrapartum period for patients with positive culture for GBS. GBS was detected in 18 patients which contributes to about 32% of the study population. The proper choice of antibiotic is important in successful disease prevention.
Study site: Obstretic and gynaecological clinic, University Malaya Medical Centre (UMMC)