Adekunle Dawodu

The profile of major congenital abnormalities in the United Arab Emirates (UAE) population.

The aim of this study was to establish the profile of major congenital malformations in the United Arab Emirates (UAE) population which has a high rate of consanguinity. All births with birth weight above 500 g in the three hospitals in the Al Ain Medical District of UAE were prospectively studied from January 1992 to January 1994. About 98% of the births in the district occur in these three hospitals. Detailed family history and clinical and relevant laboratory investigations were recorded in each case. Necropsy was not permitted. The major malformations were classified as multiple or isolated single system abnormalities as well as genetic or non-genetic disorders. Of the 16,419 births which occurred during the two year period, 173 (10.5/1000 births) had major malformations, 90 (52%) had multiple malformations, and 83 (47.97%) had involvement of a single system. Of the infants with multiple malformations, 43 had recognised syndromes, most of which are autosomal recessive disorders with a high frequency of rare syndromes. Twenty eight (31%) had chromosomal abnormalities. The most common systems involved in infants with isolated single system malformations include gastrointestinal (33), central nervous system (17), and cardiovascular (10). While the consanguinity rate was similar (57% v 54%), the frequency of first cousin marriages was much higher (51% v 30%) in the study group compared with the figures for the general population. The consanguinity rate was highest among the syndrome cases, and related parents were more likely to have infants with multiple malformations than an isolated single system abnormality with a relative risk of 1.69 (95% CL 1.27-2.24). Genetic factors could be implicated in 116 (67%) of the 173 cases of major malformations and 49 (28%) were potentially preventable. The study suggests that genetic disorders account for a significant proportion of congenital malformation in the UAE and, thus, a genetic service should be provided as part of the preventive cae programme.

Biosocial factors affecting vitamin D status of women of childbearing age in the united arab emirates

Low serum 25-OHD in female Arab subjects, which may predispose their infants to hypocalcaemia, has been suggested to be due to inadequate sunshine exposure, but may include other sociobiological factors. The effects of duration of sunshine exposure--weighted against the magnitude of clothing (UV exposure) and other sociobiological variables such as age, education and living accommodation--on serum 25-OHD and mineral status of 33 UAE national women of childbearing age were compared with those of 25 non-Gulf Arabs and seventeen Europeans. Serum concentrations of calcium, phosphorus, alkaline phosphatase and intact parathyroid hormone among the groups were not significantly different. The serum concentration of 25-OHD in UAE nationals was 8.6 ng/ml (4.5-17.4), mean +/- 1 SD, and in non-Gulf Arabs 12.6 ng/ml (6.0-26.4); both these values were significantly lower (p = < 0.0001) than the 64.3 ng/ml (49-84.3) found in Europeans. Compared with Europeans, the UAE and non-Gulf Arabs in this study were younger, had fewer years of education and had significantly lower clothing and UV scores (p < 0.0001). Furthermore, there was a positive correlation (r = 0.59425) between serum 25-OHD and UV score, but not with length of exposure. After adjusting for other confounding variables, nationality, clothing and UV scores remained major determinants of serum 25-OHD (p < 0.0001). Therefore, limited skin exposure to sunlight appears to be an important determinant of vitamin D status in our subjects. Strategies to increase vitamin D stores should include vitamin D supplementation or advice on effective sunlight exposure.

Maternal Vitamin D Status: Effect on Milk Vitamin D Content and Vitamin D Status of Breastfeeding Infants

There are increasing reports of rickets and vitamin D deficiency worldwide. Breastfeeding without adequate sunlight exposure and vitamin D supplementation are the major risk factors. In view of the drive to promote and increase the rate of exclusive breastfeeding, the relationship among maternal vitamin D status, vitamin D concentration of human milk, and hence vitamin D status of breastfeeding infants deserves reassessment. This review provides current information on the interrelationship between maternal vitamin D status and the vitamin D status of the breastfeeding infant. It also reviews the results of ongoing research on the effect of high-dose maternal vitamin D supplementation alone as a possible option to prevent vitamin D deficiency in the breastfeeding mother-infant dyad.

Risk factors and prognosis for brachial plexus injury and clavicular fracture in neonates : A prospective analysis from the United Arab Emirates

The purpose was to identify risk factors and document the incidence and prognosis for brachial plexus palsy (BPP) and clavicular fracture (CF) among consecutive hospital liveborn (LB) infants in the United Arab Emirates. During a 2-year period, all hospital-born infants were examined twice by paediatricians before discharge and those found to have BPP or CF were evaluated and followed up by an orthopaedic surgeon. Each clavicular fracture was confirmed radiologically. For the assessment of risk factors, three controls were selected for each case of BPP or CF. Of the 9231 LB, 27 (2.9/1000) had BPP while 24 (2.6/1000) sustained CF. After controlling for potential confounding variables, shoulder dystocia (SD), fetal macrosomia (birthweight > 4000 g), instrumental vaginal delivery and diabetes remained risk factors for BPP while only fetal macrosomia and instrumental delivery increased the risk of CF. The frequency of these risk factors was higher than that reported from the West. Infants with BPP had a higher incidence of SD (p = 0.0001) and tended to be heavier (p = 0.052) than those with CF. All infants with CF recovered while 20% of those with BPP had moderate to severe residual disabilities. This study highlights geographic differences in risk factors for BPP and CF and the possible contribution of BPP to morbidity in infancy. Early recognition of fetal macrosomia and improved management of SD and maternal diabetes are necessary to reduce the incidence of BPP and CF as well as morbidity from BPP in this community.

Folic acid awareness and intake survey in the United Arab Emirates.

OBJECTIVE The aim of this study was to determine the level of knowledge and use of periconceptional folic acid supplementation in a sample of postpartum women recruited from three hospitals. DESIGN Cross-sectional survey in which a structured questionnaire was used in a face-to-face encounter between the subject and a trained nurse. SETTINGS Two teaching hospitals associated with Faculty of Medicine and Health Sciences and one private hospital. SUBJECTS Postpartum women in the three hospitals were recruited during a 40-day period in November 1999. Women who did not agree to participate, had complicated labor, delivered babies with congenital malformations, or were too exhausted or difficult to examine, were excluded. RESULTS Univariate analyses showed that overall 46.4% of the respondents had heard about folic acid and only 8.7% knew that it prevented birth defects. 45.5% of respondents took folic acid in the first trimester. The percentage of women who had ever heard about folic acid was higher in those with higher education, and those who were not UAE nationals. Use of folic acid was associated with non-UAE nationality. CONCLUSION Awareness of the value of periconceptional folic acid was very low and use of folic acid was less prevalent among women of UAE nationality.

Diet, clothing, sunshine exposure and micronutrient status of Arab infants and young children

Summary Vitamin C and D levels in Arab women and their newborn infants have been shown to be low. We investigated the prevalence of and risk factors for possible hypovitaminosis C and D in a convenience sample of 51 hospitalized children without clinical features of vitamin C or D deficiency. The mean age was 15.4 months. The serum vitamin C concentration was low in the mothers but normal in the children. Both mothers and children had low serum 25-hydroxyvitamin D (25-OHD) concentrations. Fifty per cent of the mothers and 22% of the infants and children had hypovitaminosis D (serum 25-OHD level < 25 nmol/l). Infants who received dietary vitamin D supplementation had a higher mean (SD) serum 25-OHD concentration than the unsupplemented group (62.5 (29.8) vs 38.5 (27.3), p = 001). Cutaneous light exposure in these children was poor. The childrens serum 25-OHD concentration correlated with dietary vitamin D supplementation and maternal serum 25-OHD levels. The results suggest normal vitamin C status but a possible high prevalence of hypovitaminosis D in Arab children and their mothers in UAE. Health education to encourage greater sunshine exposure and improvement in maternal vitamin D stores and the availability of adequate vitamin D supplements would improve childrens vitamin D status. The study indicates that hypovitaminosis D continues to be an important maternal and child health problem, despite the abundant sunshine.

Pattern of central nervous system anomalies in a population with a high rate of consanguineous marriages

Nine thousand six hundred and ten births were prospectively studied in the three major hospitals in Al‐Ain, United Arab Emirates (UAE) between October 1995 and January 1997. Babies suspected of, or diagnosed, as having central nervous system (CNS) abnormalities were evaluated by a neonatologist, a clinical geneticist and a pediatric neurologist. Brain computerized tomography/magnetic resonance imaging (CT/MRI) was performed on all babies suspected of having CNS abnormalities. In addition, metabolic screening and chromosome analysis were also performed when indicated. Of the 225 babies with congenital anomalies identified, 31 had CNS abnormalities (3.2/1000). Syndromic abnormalities of the CNS were present in 13 cases (42%), chromosomal abnormalities in one case (3.2%) and the rest included: neural tube defect (NTD) in 11 cases (36%), holoprosencephaly in two cases (6.4%) and hydrocephalus in four cases (12.9%). Detailed analysis of the syndromic types revealed that out of the 13 cases, 12 were inherited as autosomal recessive (AR) and in one case the inheritance was undetermined. Consanguinity with high level of inbreeding was present in 12 cases and the majority of the syndromes identified were extremely rare. The study indicates that CNS anomalies are fairly common in the UAE, particularly, the recessive syndromic types. Careful and detailed analysis of such anomalies is required so that accurate genetic advice can be given.

Diversity in expression of glucose‐6‐phosphate dehydrogenase deficiency in females

The aims of this study were to determine the prevalence of glucose‐6‐phosphate dehydrogenase (G6PD) deficiency in the United Arab Emirates (UAE), to describe the different mutations in the population, to determine its prevalence, and to study inheritance patterns in families of G6PD‐deficient individuals. All infants born at Tawam Hospital, Al‐Ain, UAE from January 1994 to September 1996 were screened at birth for their G6PD status. In addition, those attending well‐baby clinics during the period were also screened for the disorder. Families of 40 known G6PD‐deficient individuals, selected randomly from the records of three hospitals in the country, were assessed for G6PD deficiency. Where appropriate, this was followed by definition of G6PD mutations. Of 8198 infants, 746 (9.1%), comprising 15% of males and 5% of females tested, were found to be G6PD deficient. A total of 27 families were further assessed: of these, all but one family had the nt563 Mediterranean mutation. In one family, two individuals had the nt202 African mutation. The high manifestation of G6PD deficiency in women may be due to the preferential expression of the G6PD‐deficient gene and X‐inactivation of the normal gene, and/or to the presence of an ‘enhancer’ gene that makes the expression of the G6PD deficiency more likely. The high level of consanguinity which, theoretically, should result in a high proportion of homozygotes and consequently a higher proportion of females with the deficiency, was not found to be a significant factor.

Neonatology in developing countries : problems, practices and prospects

A recent review of perinatal and neonatal care in developed countries found that a decline in neonatal mortality rates due to improved neonatal care has played a major role in the decline of infant mortality over the past 3 decades. However efforts to reduce high infant mortality rates (IMR) in most developing countries have focused mainly upon post-neonatal mortality while neonatal mortality (NND) has been almost ignored. A recent World Health Organization focus upon the health of newborn infants and the contribution of NND to IMR has called attention to the need to improve perinatal and neonatal care facilities and resources in order to realize any significant reduction in the high IMR observed in developing countries. One important step toward improving neonatal care and reducing neonatal mortality in developing countries is to carefully examine government health funding neonatal health care resources and the major determinants of neonatal mortality. Economic and health indicators of IMR neonatal vital statistics and causes of death and the major neonatal problems of low birth weight birth asphyxia infections and severe jaundice are discussed followed by a summary of problems and future prospects.

Epidemiology of clinical hyperbilirubinaemia in Al Ain, United Arab Emirates.

All infants born at Al Ain Hospital, United Arab Emirates between 1 January and 30 June 1995 who developed clinically relevant hyperbilirubinaemia defined as jaundice requiring investigation and treatment were prospectively studied. Of the 2300 live births, 85 (3.7%) developed hyperbilirubinaemia. Of these, 22 were premature, 22 had ABO haemolytic disease of the newborn, eight had G6PD deficiency (Mediterranean), seven had breast-milk jaundice, five were born to mothers with diabetes mellitus and one had Rh incompatibility. No specific factor was identified in 20 (24%). Significant differences in the distribution of diagnostic categories were found among the major ethnic groups in the population studied. This first study of the epidemiology of clinically relevant hyperbilirubinaemia in this community identified locally relevant risk factors and highlighted areas of health care which, if modified, might reduce the incidence of hyperbilirubinaemia.