While it is not difficult to recognise the classical clinical features of congenital syphilis in most cases, some of them may present with unusual manifestations which can defy early diagnosis. We report our experience with 13 cases of early congenital syphilis over a period of 10 years from 1980 to 1989. Twelve of the thirteen patients were less than 3 months at presentation. There were two infants born prematurely and six of the babies were born with a low birthweight (less than 2.5 kg). All but four patients survived following treatment. Skin lesions either in the form of typical vesiculobullous eruption over the palms and soles or a maculopapular skin rash over the body were the most common presentation and was seen in 10 patients. Splenomegaly with or without hepatomegaly was the most consistent physical sign. Radiological changes in the form of periostitis and/or metaphysitis were seen in all cases where an X-ray of the long bones was performed. An elevated serum immunoglobulin M, though non-specific for the disease, was found to be a useful screening test for recent infection.
Pregnancies conceived through assisted reproduction can present considerable management problems to the obstetric and paediatric staff. Multiple pregnancies are common. The complication of prematurity increases the morbidity and mortality rates of the neonates.
A preterm baby was born to a multiparous mother by emergency caesarean section at 36 weeks of gestation. Apart from a depression on the right temporo-parietal region measuring 3 cm x 3 cm x 0.5 cm, no other abnormality was noted. A CT scan of the brain excluded the presence of intracranial haematoma and pressure effect on the brain. Spontaneous reduction of the fracture without any adverse neurological sequelae suggests that these fractures can be managed conservatively in some instances.
Large ovarian cysts in the fetus are uncommon. Ultrasonography has helped in the detection of these cysts antenatally and in the newborn female infant. A case of bilateral ovarian cysts in a newborn is presented. The choice of management between conservative measures and surgical approaches remains controversial.
Most muscular dystrophies manifest as peripheral muscular weakness commencing at various age, however, oculopharyngeal muscular dystrophy (OPMD) is a rare hereditary disorder presenting in middle age with progressive dysphagia and bilateral blepharoptosis rather than peripheral muscular weakness. In the medical literature, OPMD has been well described in Canadians of French descent. So far, there is no publication of OPMD in the Malaysia-Singapore medical literature. This article documents this condition in a Chinese patient. A review of the literature is presented and the management of OPMD is discussed.
Cerebro-costo-mandibular syndrome (CCM) is a very rare entity with oro-facial features closely resembling Pierre-Robin anomaly (1,2). We describe a patient with typical features: severe micrognathia, glossoptosis, central cleft soft palate and multiple posterior rib-gap defects seen on chest radiograph. Respiratory distress which was evident soon after birth was relieved by an oro-pharyngeal airway. He suffered from a cardiorespiratory arrest and succumbed, presumably secondary to aspiration of milk feeds, before a tracheostomy could be performed.
Meconium ileus, rare in Malaysia, accounts for 3.7% of all neonatal intestinal obstructions (excluding imperforate anus) seen in the University Hospital, Kuala Lumpur, from 1980-1990. This paper retrospectively reviews our clinical experience with 5 cases of meconium ileus seen over a 12-year period from 1980-1991 in the University Hospital, Kuala Lumpur. Three of the neonates were Malays, and two were Punjabis. Four of them were full-term and one preterm. The birth weights ranged from 1900 to 3700 g, with a mean of 2670 g. One of them also had a sibling with meconium ileus. Two of them were found to have foetal ascites and one had intestinal obstruction, antenatally by ultrasonography. The remaining two were symptomatic soon after birth. Extensive calcification was observed on plain abdominal radiographs in three babies and dilated bowels in the other two. All of them underwent laparotomy and uncomplicated meconium ileus was confirmed in two cases, meconium peritonitis in two and one meconium pseudocyst in addition to meconium peritonitis. There was one intraoperative death, and one long-term survivor who did not have cystic fibrosis. The remaining three did not have additional features suggestive of cystic fibrosis, and finally succumbed to respiratory infection. Sweat test was not done to confirm the diagnosis for logistic reasons. The management of such patients proved to be a challenge to clinicians because of the rarity of this condition.
A set of twins born to a 24-year-old primigravida had evidence of sepsis 24 to 60 hours after birth and were treated empirically with penicillin and gentamicin. A non-encapsulated H. influenzae biotype IV strain was isolated from the blood cultures of both and from the CSF of twin II. The isolates were beta-lactamase positive and hence showed resistance to ampicillin and therapy was changed to chloramphenicol only. Twin II recovered but Twin I developed a brain abscess in the left occipital region which resolved with extended antibiotic treatment. Although ampicillin-resistant H. influenzae have been reported in Malaysia, invasive disease by such strains are rare.
Between February 1990 and May 1993, 13 cases of early congenital syphilis (ECS) were managed in the Paediatrics Unit, University Hospital, Kuala Lumpur. Twelve mothers were unbooked with 10 inborn babies. Only one mother had antenatal booking at this hospital but she defaulted antenatal follow-up. Several risk factors associated with ECS were identified: inadequate or no prenatal care (5/13), failure to repeat a serological test for syphilis in the third trimester when it was tested negative at first booking (5/13), sexual promiscuity, substance abuse and a past history of contracting sexually transmitted disease. All 10 mothers who had their serological test repeated at delivery were found to have a positive VDRL and TPHA. Adequate antenatal care early referral of infected, expectant mothers for treatment, and a repeat serological test for syphilis could have prevented these cases of ECS.
Extensive haemangioma with platelet consumption (Kasabach-Merritt syndrome) is rare. The lesion is usually a single cutaneous cavernous haemangioma similar to that found in our patient. Multiple superficial strawberry naevi were also seen all over the rest of the body. This condition was diagnosed antenatally in this patient with the use of colour Doppler. There is one recent report where the thrombocytopaenia was diagnosed by cordocentesis. Unfortunately the baby developed heart failure with intractable coagulopathy and died 2 days later.
Three cases of neonatal chylous ascites (CCA) were managed in the neonatal unit, University Hospital, Kuala Lumpur, over the past 9 years. Fetal ascites and polyhydramnios were the sole abnormalities detected in all three babies by antenatal ultrasonography. They were born at 36 weeks' gestation and their birth weights ranged from 3.0 kg to 3.8 kg. All three infants had abdominal distension at birth. Milky ascitic fluid was obtained after starting enteral feedings. Analysis of the ascitic fluid revealed a raised white blood cell count (predominantly lymphocytic) and triglycerides (1.4 - 3.8 mmol/l), cholesterol (1.6 - 2.8 mmol/l), and protein levels (25 - 41 g/l). Conservative management with skimmed milk and medium-chain triglycerides in one infant and Pregestimil in another was instituted. these two infants with CCA were clinically normal when reviewed at 19 months and 3.5 years of age. The third infant had a gut malrotation and associated pyloric septum; he died from complications of a laparotomy. The literature on this rare condition is reviewed.
Between January 1984 and December 1994, 30 cases of early neonatal group B streptococcus (GBS) septicaemia were managed in the Neonatal Unit, University Hospital, Kuala Lumpur. Two neonates were outborn and 28 were inborn, giving an average annual incidence of neonatal GBS septicaemia of 0.4/1000 livebirths among inborn babies. In a separate survey over a three-month period, GBS genital carriage rate among 196 parturients was found to be 9.7%. Of the infants with GBS septicaemia, the mean gestational age was 37.5 +/- 3.8 weeks and the mean birthweight was 2540 +/- 716 g. Twelve (40%) were preterm infants and 14 (47%) were low birthweight infants. Male and female infants were almost equally affected. Prolonged rupture of membranes and maternal pyrexia accounted for only 5 (17%) and 3 (10%) of the cases respectively. Twenty-four (80%) neonates had onset of symptoms within 6 hours of life and respiratory symptoms were observed in 24 (80%) of the cases, while meningitis was uncommon. Six (20%) neonates died. Preterm and low birthweight infants had higher mortality than their term counterparts: 42% versus 6% and 36% versus 6% respectively. Of those who died, 4 (67%) required respiratory support right from birth and the mean time of onset of symptoms was 4 hours (range 0 to 21 hours) and the duration of survival was only 28.8 hours (range 12 to 38 hours). As the incidence of neonatal GBS septicaemia was low, mass screening and chemoprophylaxis for GBS were not recommended. All the GBS isolates were sensitive to penicillin and ampicillin, thus one of these antibiotics should be included in the antimicrobial therapy of septic neonates.
Despite the numerous changes made in accordance with the Baby Friendly Hospital Initiative at the University Hospital, Kuala Lumpur, the low rates of breastfeeding have persisted. This study aims to examine the current trend in infant feeding, and the influences of some perinatal and sociodemographic factors on breastfeeding. Five-hundred mothers with singleton pregnancies and healthy infants were interviewed at 6 weeks post-partum. Only 124 (25 per cent) mothers were practising exclusive breastfeeding (EBF), and 132 (26 per cent) mothers were using exclusive infant formula feeding (EIF). On logistic regression analyses, mothers who followed EBF were more likely to have had antenatal plans to breastfeed (Odds ratio 2.44, 95 per cent confidence interval 1.75-3.45), not in paid employment post-natally (OR 1.76, 95 per cent CI 1.31-2.36), of older age group (> 27 years) (OR 1.48, 95 per cent CI 1.13-1.93), had female infants (OR 1.38, 95 per cent CI 1.05-1.80) and of Indian ethnicity (compared to Chinese) (OR 3.87, 95 per cent CI 2.16-6.89). Breastfeeding difficulties were associated with decreased odds of EBF (OR 0.21, 95 per cent CI 0.13-0.34). Parental education, fathers' ages and incomes, primigravida status, Caesarean section, present of episiotomy, late first breastfeed, phototherapy, and length of hospital stay were not significant predictors of failure of EBF. In comparison, predictive factors for increased use of EIF were mothers who have had breastfeeding difficulties, < or = 9 years of schooling, and of Chinese descent. In conclusions, the overall rate of EBF by 6 weeks of age in infants born in this urban hospital had remained poor. The adverse factors for EBF identified in this study warrant further in-depth studies to determine effective ways of improving EBF rates.
Cholestatic disorders of infancy (viz neonatal hepatitis and biliary atresia) have not been well studied in Malaysia. In a retrospective study in the Department of Paediatrics, University Hospital, Kuala Lumpur from January 1982 through December 1991, a total of ninety-three infants with such conditions were identified: 35 (38%) had biliary atresia, 58 (62%) neonatal hepatitis. There was a statistically significant male preponderance in the neonatal hepatitis group (P = 0.020). There was no significant difference in the racial distribution and in the proportions of low birthweight infants between the two groups of disorders. When the biliary atresia group was compared with the neonatal hepatitis group, significant differences were observed in the age of presentation (mean +/- SD) 9.8 +/- 6.8 VS 20 +/- 17.3 weeks (P < 0.001), proportion of infants with prolonged jaundice (> seven weeks) 28/35 (80%) VS 20/58 (34.5%) (P < 0.00001), occurrence of alcoholic stools 26/35 (74.3%) VS 27/58 (46.6%) (P = 0.020), liver size (mean +/- SD): 4.3 (1.6 cm VS 3.3 +/- 1.8 cm (P < 0.01) and splenic size: 2.5 (1.8 cm VS 1.4 (1.2 cm (P < 0.001). There was however considerable overlap between the two groups in these features at presentation, making clinical differentiation between the two conditions difficult. Infants with cholestasis tended to present late, compromising the chance of survival. In order to improve the medical care of these patients, these conditions must be emphasised during the training of medical practitioners, and efforts to increase public awareness of these conditions must be created.
Dengue, an important mosquito-borne flavivirus infection, is endemic in Southeast Asia. We describe two mothers who had acute dengue 4 and 8 days before the births of their infants. One mother had worsening of her proteinuric pregnancy-induced hypertension, liver dysfunction, and coagulopathy and required multiple transfusions of whole blood, platelets, and fresh frozen plasma. Her male infant was ill at birth, developed respiratory distress and a large uncontrollable left intracerebral hemorrhage, and died of multiorgan failure on day 6 of life. Dengue virus type 2 was isolated from the infant's blood, and IgM antibody specific to dengue virus was detected in the mother's blood. The second mother had a milder clinical course; she gave birth to a female infant who was thrombocytopenic at birth and had an uneventful hospitalization. Dengue virus type 2 was recovered from the mother's blood, and IgM antibody specific to dengue virus was detected in the infant's blood. This report highlights not only the apparently rare occurrence of vertical transmission of dengue virus in humans but also the potential risk of death for infected neonates.
A prospective study was carried out at the University Hospital, Kuala Lumpur to determine the cervical carriage rate of Ureaplasma urealyticum and Mycoplasma hominis among healthy pregnant women at delivery and the incidence of nasopharyngeal colonisation among their infants.
Thirty preterm infants weighing > or = 800 g with clinical and radiological evidence of respiratory distress syndrome (RDS) requiring mechanical ventilation with FiO2 of > or = 40% were given modified bovine surfactant (Survanta). They were compared with equal number of historical controls. Infants who received surfactant showed prompt and highly significant improvement in FiO2, mean airway pressure, arterial/alveolar oxygen tension ratio and ventilatory index. There was significant improvement in mortality rate (10% vs 33%; p = 0.03). Among the survivors, surfactant-treated infants required shorter duration of continuous positive airway pressure (CPAP) (3.4 vs 9.6 days; p = 0.04). For survivors with birthweight of > 1000 g, surfactant-treated infants required shorter duration of ventilatory support (intermittent positive pressure ventilation + CPAP) (7.5 vs 18.9 days, p = 0.02). Overall, surfactant-treated infants achieved full enteral feeds sooner (15.7 days vs 24.6 days; p = 0.03) and required shorter duration of total parenteral nutrition (13.9 days vs 25.6 days; p = 0.02). We concluded that surfactant replacement therapy was effective in the treatment of preterm infants with RDS.
AIMS: To examine the pattern of and the influence of some socio-demographic factors on infant milk feedings, and the protective role of breastfeeding against infections.
METHODS: Mothers who breastfed their infants (exclusively or partially) at 6 weeks postpartum, and who had singleton pregnancies and healthy infants at birth, were interviewed when their infants had reached 6 months of age.
RESULTS: Of the 234 mothers studied, only 31 (13%) mothers were practising exclusive breastfeeding (EBF) and 133 (57%) mothers were using exclusive infant formula feeding (EIF). Solid and semi-solid foods were introduced between 4 to 6 months of life in 89% of the infants. On logistic regression analysis, mothers who were in paid employment [OR 0.25, 95% CI 0.15, 0.42] and not breast feeding at 6 weeks [OR 0.32, 95% CI 0.19, 0.54] had decreased odds of EBF. Antenatal plans to breastfeed, breast-feeding difficulties, ethnicity, level of parental education, parental ages, fathers' income, primigravida status and infants' gender were not significant co-variates. In comparison, EIF was more likely in mothers who worked, practised mixed feedings at 6 weeks and of Chinese descent. There were no significant differences in the rates of upper respiratory tract infections (URTI) or diarrhoeal illnesses between the infants who were or were not being breast-fed.
CONCLUSIONS: Most mothers were unable to breastfeed their infants exclusively in the recommended first 4 to 6 months of life. Complementary changes outside the hospital and maternity services are essential in improving breastfeeding rates. Breastfeeding does not appear to confer significant protection to either URTI or gastrointestinal tract infections.
Study site: Postnatal clinic, University Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia