Failure to screen for sickle cell disease at the University Hospital of the West Indies

OBJECTIVE: Universal neonatal screening for sickle cell disease using cord blood has taken place in the University Hospital of the West Indies (UHWI) since 1997. Comprehensive quality control of the screening programme has not been implemented. Our aim was to determine how many deliveries were left unscreened, and to ascertain factors contributing to failure to screen for sickle cell disease. METHODS: All live births in 1999 entered in the delivery book of the Labour Ward, UHWI, were scrutinized against the sample book of the Neonatal Screening Laboratory, Sickle Cell Unit. Identified unscreened infants were matched with a randomly chosen control, born on the same day. Time and mode of delivery, persons assisting the delivery, birth weight, level of urgency, location of delivery and Apgar scores at 1 and 5 minutes were collected for unscreened infants and screened controls. Secular variation within the year was assessed by comparing the frequency of unscreened births in each month to the previous month. Frequencies of potential determinants of failure to screen were tabulated. The independent predictive power of these potential determinants was assessed using conditional logistic regression, to account for correlations between predictors, and for the case-control design of the study. RESULTS: Of 2,763 live births in 1999, 139 (5 percent) infants were not screened for sickle cell disease. There was no statistically significant secular variation within the year (relative risk 1.02, 95 percent confidence interval 0.97, 1.07, x2 = 0.77, p = 0.38). Independently significant predictors of failure to screen were: emergency delivery (odds ratio 4.8, 95 percent confidence interval 2.3, 10.0), nigttime delivery (OR2.5, 0.9) CONCLUSIONS: This study identified that 5 percent of infants born in UHWI in 1999 were discharged from hospital without having been screened for sickle cell disease. Statistically significant associations with failure to screen were found to be emergency delivery, night-time delivery, and low 5 minute Apgar score. The risk of being left unscreened increased 5-fold for emergency deliveries. Increased awareness of these risk factors may reduce failure to screen. Of course, such effort does not obviate the need for regular quality control, including timely identification and tracing of unscreened infants. (AU)

Evaluation of neonatal sepsis screening in a tropical area. Part III: neonatal sepsis in meconium stained deliveries

Of the 6,060 consecutive live births delivered at the University Maternity Unit of Guadeloupe (French West Indies) during a 30-month period, 635 newborns (10.4 percent) presented with meconium stained (MS) amniotic fluid, of which 595 (94 percent) received bacteriological screening at birth (light MS, n=543; thick MS, n=52). Thirty (5 percent) of MS newborn had a bacteraemia (n=13, group B streptococcus, GBS), and 128 (21.5 percent) a bacterial positive gastric aspirate (n=54, GBS). Sixty-six newborns among MS babies needed tracheal suctioning (11 percent) in the delivery room for meconium inhalation. Among these 595 screening MS newborn, 286 (48 percent) presented clinical signs of postmaturity of birth, having therefore an explanation for their MS condition. For the other MS newborn without the postmaturity explanation, we experienced twofold increased risk of neonatal sepsis (OR 1.88 for bacteraemia and 2.61 for external carriage p < 0.02, Chi square) as compared with their MS postmature counterparts. We conclude that when meconium stained deliveries are associated with postmaturity signs, one may not need to initiate prophylactic antibiotic treatment at birth unless they present with other traditional risk factros for neonatal sepsis such as intrapartum fever and prolonged rupture of membranes.(Au)

Evaluation of neonatal sepsis screening in a tropical area. Part II: Evaluation of intrapartum chemoprophylaxis protocol

The authors report on an analysis of a chemoprophylaxis protocol at the University Hospital of Guadeloupe in the Caribbean. This study comprised 6,060 consecutive deliveries and was initiated to assess the application of an intrapartum chemoprophylaxis protocol, evaluate the results, and try to identify possible necessary modifications to the existing protocol. Although more than 90 percent of women had at least one bacterial screening (vaginal or urinary) during the last trimester of pregnancy, approximately 75 percent of mothers who were heavily colonized group B streptococcus (GSB) at delivery were not detected by this systematic screening. As is also reported in other tropical areas where a great portion of nenonatal sepsis occurs in term babies, low birthweight was not a specific risk factor in this study when controlling for other major risk factor such as fever and premature rupture of membranes. Intrapartum chemoprophlaxis was associated wiyh an approximate three fold decrease in the risk of GBS neonatal bacteraemia among at risk deliveries. The results suggest that, in our tropical context, prolonged rupture of membranes of at least 12 hours' duration should be considered as a cause for intrapartum chemopropylaxis as it accounted for the majority of neonatal bacteraemia that escaped the existing protocol. (AU)

Neonatal sepsis and mortality in a regional hospital in Trinidad: aetiology and risk factors

A total of 132 neonatal deaths among 627 infants admitted to the neonatal ward of the San Fernando General Hospital, Trinidad over a 2-year period were reviewed. The most common cause of death was prematurity (43.9%). Infection was the second most common cause (21.2%). Pseudomonas aeruginosa and Staphylococcus aureus were the most frequently isolated organisms (43%). The major drugs used empirically in suspected cases of sepsis were ampicillin or ceftazidime plus gentamicin. About 85% of S. aureus were resistant to ampicillin, and P. aeruginosa resistance to ceftazidime and gentamicin was 76.7% and 72.1%, respectively. Significant risk factors in maternal history were infrequent antenatal care and prolonged rupture of membranes. The incidence of infection among low birthweight infants was 85.6%. Early-onset sepsis (86.4%) seemed to have a nosocomial origin because of the type of pathogens seen. There is an urgent need to improve the staff-to-patient ratio in the neonatal unit and for staff to be constantly reminded to employ simple infection control practices such as proper hand-washing to reduce cross-infections.

Evaluation of neonatal sepsis screening in a tropical area. Part I: Major risk factors of bacterial carriage at birth in Guadeloupe

This prospective study reports on screening for neonatal sepsis among 3,372 live births out of 6,060 consecutive deliveries at the University Hospital of Pointe-a-Pitre, Guadeloupe, during a 30 month period. Group B Streptococcus (GBS) was the most common pathogen, representing 46 percent (89/194) of positive blood cultures and 52 percent (335/637) of positive gastric aspirates. Although only 3,372 (55 percent) of all live births were screened, 637 (10 percent) had gastric bacterial carriage at birth; of those, 335 (5.5 percent) involved GBS. Similarly, there were 194 (3.2 percent) positive blood cultures, of which 89 (1.5 percent) involved GBS. In this report, all newborns who presented with a positive GBS blood culture had at least one of the external tests positive for GBS (gastric, ear canal, rectum and placenta). Thirty-seven per cent (14/38) of positive neonatal blood cultures occurred in newborns with foetid liquor while in deliveries with intrapartum fever 16.5 percent (32/195) of blood cultures were positive. In our clinical practice, characteristics that were evident in the delivery room (without knowledge of prenatal follow-up) such as foetid liquor, intrapartum fever, prolonged rupture of membranes, foetal tachycardia and meconium staining were associated with the great majority of neonatal sepsis.(Au)

Our experience with Retinophaty of prematurity screening in Curacao

At the beginning of 1996, we started with a structural periodical screening of babies that were prematurely born. This was done utilizing a protocol that was developed in cooperation with both the author and the Neonatology Department of our hospital in Curacao. After more than 200 screenings only one case of retinopathy of prematurity, stage 3, was detected. (AU)

Neonatal Screening for Haemoglobinopathies in South East London: findings and comparisons with Jamaica

INTRODUCTION: Neonatal screening for sickle cell disorders has been shown to reduce mortality and morbidity. Methods of screening vary but in 1994, the local Health Authority funded universal neonatal screening across the whole of Lambeth, Southwark and Lewisham. This paper will report the findings of the first three years of operation of the programme and compare findings with the screening programme currently operating in Jamaica. METHOD: since May 1994 dried bloo[d] spots of all infants have been screened at King's College Hospital and screen positive cases followed up by counsellors. Infants are followed up at four sites (Guy's, King's, Lewisham and St.Thomas') according to parental preference. Minimum standards for follow-ups have been agreed by clinicians across all four sites and information of the success in achieving these standards is now being collected. RESULTS: Overall there have been 122 affected infants detected in the first two years 10 months of the programmes operation. This is made up of 83 HbSS, 35 HbSC, 4HbSBThal. This gives a birth preference of 23.4 per 1000 total population (2.3HbSS, 1.0 HbSC). Allowing for a termination rate of 20 percent this indicates that the expected birth prevalence in the district would be 4.2 per 1000 births. This compares with birth prevalence of of 0.3 per 1000 for congenital hypothyroidism, 06. per 1000 for cystic fibrosis and 0.1 for phenylketonuria. The distribution of the births is unevem with 57 in Southwark, 40 in Lambeth and 25 in Lewisham. The paper will report on the follow-up and outcome of care provided for this population to date. Discussion: South East London has the highest prevalence of sickle cell disorders of any district in the UK. Sickle cell disorder is now as common in South East London as it is in Jamaica. The follow-up arrangements established in South East London provide an opportunity for colloboration with the West Indies which may help to determine some of the reasons for differences in the natural course of the disease in these populations. The population based approach established should allow monitoring of the impact of community education and antenatal screening programmes on the birth prevalence over time. (AU)

The findings of a London wide needs assessment for sickle cell dis orders

The size of the population in London with Sickle cell disorders has increased markedly in the past 20 years and it is now one of the commonest reasons for admission of children to hospital in inner London. This paper presents the findings of a London Wide Needs assessment for sickle cell disorders.It reviews Health and Local Authorities response to the needs of their populations in terms of the development of strategy and services. It also considers reasons for the failures to develop appropriate services[AU]

Screening of cord blood for haemoglobinopathies and thalassaemia by high-performance liquid chromatography - abstract

A high-performance liquid chromatography (HPLC) method for the screening of haemoglobins in cord blood was evaluated and the gene frequencies of the structural haemoglobin variants HbS and HbC and the synthesis variants O- and á+ -thalassaemia were studied in babies born on the Caribbean island of Curacao, the Netherlands Antilles. In 3 months, 67.2 percent of all (748) newborns were screened and 122 (24.3 percent) had an abnormal haemoglobin pattern, of which 53 (43.4 percent) had a haemoglobinopathy (HbS or HbC), 64(52.2 percent) O-thalassaemia (Hb Barts > 0.5 percent, corresponding with heterozygous or homozygous O-thalassaemia-2) and 5 (4.1 percent) a haemoglobinopathy plus O-thalassaemia. None of the newborns with heterozygous HbS and HbC had concomitant á+-thalassaemia. The population genotype frequency of heterozygous O-thalassaemia -2 remain undetected. The data are in excellent agreement with comparable published results. The HPLC method proved pre-eminently suitable for the screening of cord blood samples. (AU)