The MSC differs significantly from cohorts based in the USA, UK or Jamaica due to the near 100% of

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The MSC differs significantly from cohorts based in the USA, UK or Jamaica due to the near 100% of patients with HbSS, compared to the HbSC observed in diaspora populations of a West African origin (with its milder clinical course and lower mortality rate). Additionally a very high proportion of Tanzanian SCD patients (>90%) (Makani et al. unpublished data) have the Central African Republic (Bantu) haplotype associated with more severe disease and end-organ damage [34] compared to the Senegal, Benin or Arab haplotypes. This more severe sub-type of SCD strengthens the need for intervention and offers an appropriate location for this trial.
Malnutrition in MSC
The growth charts in Appendix 2 illustrate the profound growth retardation of
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Markers of iron status suggest a lower prevalence of iron deficiency in the MSC compared to local controls: transferrin saturation <16% = 25% [N=835] vs 47%[N=79] in controls. Moreover, our data suggest that higher iron status (but not iron overload) assessed by transferrin saturation is associated with lower daytime and nocturnal haemoglobin oxygen saturations [36]. Transferrin saturation is not associated with haemoglobin concentrations in SCD cases, compared to a strong correlation in the non-SCA controls, whilst adequate iron status assessed by the F-index (ratio of soluble transferrin receptor to logged ferritin), is paradoxically associated with greatly increased odds of having averaged steady state haemoglobin concentrations in the lowest septile (RR=5.45 [2.71/10.96] P<0.001). Hence iron will not be included in the fortificants of the current intervention.
Amino acid status in MSC
In a small pilot nested-case-control study, we assessed plasma amino acids in stored steady-state plasma samples from 11 SCD patients who had died (age at death 20.9±7.4y) compared to 12 matched survivors. In confirmation of results from adult patients in the USA [21], we found significantly lower ratios of arginine to ornithine (0.51±0.14 vs 0.68±0.17, p=0.014) in the patients who died [37]. Furthermore, plasma arginine and arginine:ornithine ratios were significantly lower in our SCD patients compared to non-sickle children from Dar-es-Salaam
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