One-third of the new paediatric patients with sickle cell disease in The Netherlands are immigrants and do not benefit from neonatal screening

Abstract

One-third of the new paediatric patients with sickle cell disease in The Netherlands are immigrants and do not benefit from neonatal screening Peters, M.; Fijnvandraat, C.J.; van den Tweel, X.W.; Garre, F.G.; Giordano, P.C.; van Wouwe, J.P.; Pereira, R.R.; Verkerk, P.H. Published in:Archives of disease in childhood DOI: 10.1136/adc.2009.165290 Link to publication Citation for published version (APA): Peters, M., Fijnvandraat, C. J., van den Tweel, X. W., Garre, F. G., Giordano, P. C., van Wouwe, J. P., ... Verkerk, P. H. (2010). One-third of the new paediatric patients with sickle cell disease in The Netherlands are immigrants and do not benefit from neonatal screening. Archives of disease in childhood, 95(10), 822-825. https://doi.org/10.1136/adc.2009.165290 General rightsIt is not permitted to download or to forward/distribute the text or part of it without the consent of the author(s) and/or copyright holder(s), other than for strictly personal, individual use, unless the work is under an open content license (like Creative Commons). Disclaimer/Complaints regulationsIf you believe that digital publication of certain material infringes any of your rights or (privacy) interests, please let the Library know, stating your reasons. In case of a legitimate complaint, the Library will make the material inaccessible and/or remove it from the website. Please Ask the Library: http://uba.uva.nl/en/contact, or a letter to: Library of the University of Amsterdam, Secretariat, Singel 425, 1012 WP Amsterdam, The Netherlands. You will be contacted as soon as possible. Results The prevalence of SCD in children living in The Netherlands on 1 January 2003 was 1:5152 (95% CI 1:4513 to 1:6015). In the next 4 years, the annual incidence was 1:2011 (95% CI 1:1743 to 1:2376). Nearly one-third (27%) of the children newly diagnosed as having SCD immigrated to The Netherlands after birth and would, therefore, be missed by the neonatal screening programme. Approximately 60% of all children with SCD were not reported by paediatricians. Conclusion The number of children with SCD in The Netherlands is much higher than previously estimated, and the majority of these children seem not to be reviewed regularly by a paediatrician. Children born abroad (27% of new cases) do not benefi t from neonatal screening and are at high risk of life-threatening complications before SCD is diagnosed. As this introduces disparities in healthcare, the initiation of adequate measures should be considered.In 2007, a national neonatal screening programme for sickle cell disease (SCD) was launched in The Netherlands, prompted by the increase of the population at risk from 0.5 to 1.7 million people over the last two decades. 1 SCD is an inherited disorder of haemoglobin with its highest incidence in people of African ancestry and an increasing prevalence in Europe. 2 Presently, SCD is the most common genetic disorder in the UK and is increasingly common in The Netherlands, with a carrier incidence of 0.4%. 3-5 SCD is characterised by chronic haemolytic anaemia...

The health of migrant children in Switzerland

“…High rates of tuberculosis and intestinal parasites found in paediatric asylum seekers in Geneva (Manzano and Suter 2002) reflect imported health needs linked to different prevalence of disease in countries of origin. International reports of tropical diseases (Moudgil and Kosut 2007), and of increased rates of certain infectious diseases (MacPherson et al 2006) in migrant children and a different pattern of congenital diseases [e.g., sickle cell disease in African children (Peters et al 2010)] illustrate, that paediatricians need to consider affections uncommon in central Europe, varying with the country of origin, in migrant children, even if some of these affections have not yet specifically been researched among immigrant children in Switzerland.…”

Strategies for basic laboratory diagnostics of the hemoglobinopathies in multi‐ethnic societies: interpretation of results and pitfalls

“…However, iron deficiency does not reduce the elevated HbA2 levels of the b-thalassemia carrier to normal [38]. 4 The HbA2 measurement is of no diagnostic significance in the presence of both HbA and any other common b-variant (HbS, C, E, D, etc.). The presence of both major hemoglobin fractions indicates that both b-genes are expressed and herewith is the presence of a (nonexpressed) b-thalassemia gene excluded.…”

“…The RDW is however altered in several cardiac and hepatic conditions [22] and may have a limited discriminating potential, especially in case of combined iron deficiency. 4 Complete blood count and RCM in hemoglobin Variants: HbS, C and D usually have normal CBC values unless associated with a-thalassemia. HbS (a b-gene defect) and a-thalassemia are on different chromosomes and therefore independently inherited but, as they both are very frequent traits in Africans and Asians, combinations of HbS with a-thalassemia are very common in these populations.…”

“…Severe forms, such as b-thalassemia major (TM) and sickle cell disease (SCD), manifests gradually a few months after birth, and although a correct diagnosis can be dangerously delayed, especially in nonendemic areas [4], severely affected children rarely escape a provisional clinical diagnosis. Nevertheless, the clinician still needs laboratory assistance for confirmation at the basic and at the molecular levels for genotype-phenotype correlation, prognosis, tailored treatment, and primary prevention for any subsequent children the couple may choose to have [5].…”

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“…Adults with SCD, however, do not receive the same mortality benefit from newborn screening, penicillin and immunizations. Immigration of unscreened persons may alter data analysis, but it is postulated that the mortality burden of SCD has shifted towards later years [7,11]. Heightened awareness is therefore essential to provide effective management of adults with SCD and fever.…”