Current maternal-infant micronutrient status and the effects on birth weight in the United Arab Emirates.

Micronutrient deficiencies exist among women of childbearing age in the United Arab Emirates but the effects of maternal micronutrient deficiency on fetal growth are not well documented. To investigate the association between micronutrients and birth weight, we measured maternal and cord blood micronutrients (vitamin A, C, D, and E) and ferritin in 84 term, singleton infants born to healthy Arab and South Asian women at Al-Ain hospital. Median serum ascorbic acid and 25-hydroxyvitamin D (25-OHD) concentrations were low in mothers and infants. In multivariate analysis, maternal serum 25-OHD correlated positively with birth weight while serum ferritin showed a negative correlation.

Current maternal-infant micronutrient status and the effects on birth weight in the United Arab Emirates

Micronutrient deficiencies exist among women of childbearing age in the United Arab Emirates but the effects of maternal micronutrient deficiency on fetal growth are not well documented. To investigate the association between micronutrients and birth weight, we measured maternal and cord blood micronutrients [vitamin A, C, D, and E] and ferritin in 84 term, singleton infants born to healthy Arab and South Asian women at Al-Ain hospital. Median serum ascorbic acid and 25-hydroxyvitamin D [25-OHD] concentrations were low in mothers and infants. In multivariate analysis, maternal serum 25-OHD correlated positively with birth weight while serum ferritin showed a negative correlation

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

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 children's 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 children's vitamin D status. The study indicates that hypovitaminosis D continues to be an important maternal and child health problem, despite the abundant sunshine.

Disease severity associated with cystic fibrosis mutations deltaF508 and S549R(T-->G).

We compared the clinical severity associated with the two cystic fibrosis (CF) mutations S549R(T-->G) and deltaF508. Clinical and biochemical variables of CF were compared in two age- and sex-matched groups of CF children in the United Arab Emirates (UAE). The clinical severity of mutations S549R(T-->G) and deltaF508 showed comparable patterns, with very low Shwachman scores and high sweat chloride levels. We conclude that patients homozygous for the CF mutations deltaF508 and S549R(T-->G) have a severe clinical presentation and illness and are indistinguishable on clinical grounds.

Rational prescribing for childhood pneumonia.

The aim of this study was to assess the management and prescribing patterns for community acquired pneumonia in children in a provincial hospital setting and, further, to discover the evidence on which treatment choices were made and ascertain the need for management guidelines. The method employed was to obtain the relevant data by a retrospective audit of the case notes of children admitted with pneumonia to Wairau Hospital, Blenheim, New Zealand. The findings indicated that there were 12 different treatment regimens employed. There appeared to be no rational basis for the choices made and no microbiological evidence to support the frequent use of broad spectrum antibiotics. No difference in clinical efficacy was found between the main regimens used. It is concluded that a simple management protocol for childhood pneumonia using a narrow spectrum antibiotic initially would be as effective, more logical and cheaper.

Disease severity associated with cystic fibrosis mutations deltaF508 and S549R[T-G]

We compared the clinical severity associated with the two cystic fibrosis [CF] mutations S549R[T[R]G] and deltaF508. Clinical and biochemical variables of CF were compared in two age- and sex-matched groups of CF children in the United Arab Emirates [UAE]. The clinical severity of mutations S549R[T[R]G] and delta F508 showed comparable patterns, with very low Shwachman scores and high sweat chloride levels. We conclude that patients homozygous for the CF mutations deltaF508 and S549R[T[R]G] have a severe clinical presentation and illness and are indistinguishable on clinical grounds

The geographic distribution of cystic fibrosis mutations gives clues about population origins.

UNLABELLED: Information regarding three of the more common cystic fibrosis mutations is presented (delta F508, G542X, N13031K) to support the concept of a geography associated with cystic fibrosis mutations. We present the hypothesis that a knowledge of the geography of cystic fibrosis mutations is important for an understanding of genotype-phenotype correlations, gene flow, historical population migration and cystic fibrosis screening. CONCLUSION: A new method of study of mankind's cultural spread is being revealed and the survival of the various mutations supports the concept that they may provide a selective advantage to the carrier.

Group A streptococcal throat colonization is a dynamic phenomenon.

In order to gain knowledge about the nature of group A streptococcal throat colonization of asymptomatic children, we studied a cohort of healthy children selected at random. Those with throat cultures positive for group A Streptococci (GAS) were followed for 5 weeks. Repeat throat swabs were obtained at weekly intervals for antigen detection and culture. GAS obtained were serotyped for T antigen. Twenty-two children were identified as having GAS and 50% had a positive antigen test and culture at least five times in 6 weeks. There was no consistent pattern in the typeable organisms. Most individuals had more than one T-type alternating with non-T-typeable organisms. We conclude that colonization may be a dynamic process involving numerous organism serotypes, which wax and wane.

Bartter syndrome: an overview.

The term Bartter syndrome denotes a group of renal diseases which share a common denominator of hypokalaemia and metabolic alkalosis. The patch-clamp technique has made possible the analysis of single ion channels, improving our understanding of the molecular physiopathology of all the 'Bartter-like' syndromes. Genetic mapping of each defect has further clarified the mutations involved and the possible modes of inheritance. This improved understanding has opened new avenues for therapy, improving mortality and morbidity in these patients. Another group of illnesses, the 'pseudo-Bartter syndrome', may produce a hypokalaemic metabolic alkalosis without primary renal disease. The underlying illness needs to be identified and treated.

Complex allele [-102T>A+S549R(T>G)] is associated with milder forms of cystic fibrosis than allele S549R(T>G) alone.

We recently reported a novel complex allele in the cystic fibrosis transmembrane regulator (CFTR) gene, combining a sequence change in the minimal CFTR promoter (-102T>A) and a missense mutation in exon 11 [S549R(T>G)]. Here we compare the main clinical features of six patients with cystic fibrosis (CF) carrying the complex allele [-102T>A+S549R(T>G)] with those of 16 CF patients homozygous for mutation S549R(T>G) alone. Age at diagnosis was higher, and current age was significantly higher (P=0.0032) in the group with the complex allele, compared with the S549R/S549R group. Although the proportion of patients with lung colonization was similar in both groups, the age at onset was significantly higher in the group with the complex allele (P=0.0022). Patients with the complex allele also had significantly lower sweat test chloride values (P=0.0028) and better overall clinical scores (P=0.004). None of the 22 patients reported in this study had meconium ileus. All 16 patients homozygous for S549R(T>G), however, were pancreatic insufficient, as compared with 50% of patients carrying the complex allele (P=0.013). Moreover, the unique patient homozygous for [-102T>A+S549R(T>G)] presented with a mild disease at 34 years of age. These observations strongly suggest that the sequence change (-102T>A) in the CFTR minimal promoter could attenuate the severe clinical phenotype associated with mutation S549R(T>G).

Radiological analysis of children with cystic fibrosis who are homozygous for cystic fibrosis transmembrane conductance regulator mutation S549R (T-->G).

Genotype-phenotype analyses in cystic fibrosis (CF) have shown that cystic fibrosis transmembrane conductance regulator (CFTR) genotypes can predict pancreatic status but that correlations with pulmonary status remain elusive. We investigated the extent and severity of lung disease associated with CFTR mutation S549R (T-->G). This mutation is localized in intron 11 (nucleotide-binding fold 1 of the CFTR protein) and had so far been described as a private mutation only. It is associated with an extremely severe overall CF phenotypic expression. Detailed radiological analyses were performed by a single observer in 12 children with CF from the United Arab Emirates who were homozygous for CFTR mutation S549R (T-->G). A diversity of pulmonary changes included marked hyperinflation in early infancy in conjunction with inflammation of the interstitium. After 2 years of age, signs of central airway involvement occurred in association with early signs of pulmonary hypertension. In conclusion, although there is some diversity in the radiological findings of these CF patients, R549 is a very severe allele associated with extreme lung disease and rapid pulmonary decline.

Safer roads for children in the United Arab Emirates.

A study was conducted to obtain a representative view of the parents of primary school children regarding which strategies should be employed to reduce the toll of road traffic accidents in the United Arab Emirates. A cross-sectional population-based study was conducted in Al-Ain, UAE, in which parents were asked to give their suggestions on how to make the roads safer for their children. Strategies aimed at reducing vehicle speed, improved personal driving, adherence to existing rules, and driver and pedestrian education were regarded as paramount. There is widespread awareness of road safety problems in the country and the public appears ready to accept major safety initiatives by government agencies.

Genotype-phenotype correlations in cystic fibrosis: clinical severity of mutation S549R(T-->G).

With a view to assessing genotype-to-phenotype correlations in cystic fibrosis (CF), the clinical presentation of CF children from the United Arab Emirates (UAE) who were homozygous for cystic fibrosis transmembrane conductance regulator (CFTR) mutation S549R(T-->G was investigated. This mutation is localized in intron 11 (nucleotide binding domain 1 of the CFTR protein) and had so far been described as a private mutation only. The associations between the R549/R549 genotype and 20 outcome variables, including age at diagnosis, sweat chloride concentrations, growth percentiles, meconium ileus, pancreatic sufficiency, pulmonary disease, associated complications and micro-organism colonization were examined in a group of 15 CF children (9 females and 6 males). Mean current age and age at diagnosis were both low (5.4+/-3.5 and 1.0+/-1.1 yrs, respectively). Although none of the 15 CF patients had presented with meconium ileus at birth, all were pancreatic insufficient and had very severe lung disease, with a high rate of Pseudomonas aeruginosa and Staphylococcus aureus. Two patients died during the course of this investigation (one was 5 months and the other, 6 yrs old). The clinical presentation associated with S549R(T-->G) homozygosity in the United Arab Emirates is quite homogeneous and shows an extreme degree and course of cystic fibrosis severity.

Safer roads for children in the United Arab Emirates

A study was conducted to obtain a representative view of the parents of primary school children regarding which strategies should be employed to reduce the toll of road traffic accidents in the United Arab Emirates. A cross-sectional population-based study was conducted in Al-Ain, UAE, in which parents were asked to give their suggestions on how to make the roads safer for their children. Strategies aimed at reducing vehicle speed, improved personal driving, adherence to existing rules, and driver and pedestrian education were regarded as paramount. There is widespread awareness of road safety problems in the country and the public appears ready to accept major safety initiatives by government agencies