For severaldecadesit has been recognizedthat childrenwithsicklecell disease(SCD), especiallythosewithHbSSgenotypeSCD (SCD-SS), have poorgrowthanddelayed maturation.In addition,childrenwith$CD experiencefrequentSCD-reiated painandfever episodesand alsoinfection. Increasednutrientrequirementsand/orpoornui/;iJonalstatushave been documentedin childrenwithSCD suggestingthat chronicundemutritionmaycontributeto poorhealthoutcome,growthfailureand delayeddevelopment.Low serumlevelsof vitaminAand vitaminBehavebeen documentedinchildrenwithSCD suggestingthatdietary intakeof thesemicronutribentsmay notbe adequ._ tomeet the increasednutrientneedsof childrenwith SCD. The proposedstudyconsistsof twoprojectsto determinewhether supplementationof vitaminAor vitaminB6,,can improvehealth outcomes,nutritionalstatus,growthand hematologicstatus in childrenwithSCD-SS.Our preliminarydata fromprepubertalchildrenwithSCD showthat66% havesuboptimalvitaminA status(serumretinol<30 pg/dL),and that thosechildrenwithsuboptimalstatushavemore frequenthospitalizations, have reducedbodymassindex,and poorerhematologicstatusthan those withnormalvitaminA status. Furthermore,preliminarystudiesinchildrenandadolescentswithSCD showthat suboptimalvitamin Bestatus(serumpyridoxal5'-phosphate[PLP]< 20 nmol/L)isalsoprevalent(77%), andthat low serumBeconcenltationis associatedwithpoornutritionalstatus as indicatedbyreducedbodymassindex,weightand mid-armcircumferenceandalsowithpoorerhematologicstatus. The firststudyis arandomizedplacebo-controlclinicaltrialto determinethe effectof vitaminA supplementationat thecurrentRecommendedDietaryAllowance(RDA) on numberof hospitalizations,the numberand lengthof both SCD-related and nonSCD-relateddiseaseevents, on growth,bodycomposition,hematologicstatus,rod cell integrity,and immunestatus in prepubedalchildren(ages 2.0 to 9.9 years) withSCD. Seventy-five childrenwithSCD will be screenedforvitaminA status and44subjectswith serumretinollevels< 30 pg/dLat screeningwillberandomizedto receiveeitherthe RDA for vitaminA dailyor placebofor 12 months. The secondstudywill explore the effectof vitaminB, supplementationat twodoseson growthand nutritionalstatus and hematologicalindicesofSCD diseaseseverityinchildrenand adolescents (ages 6.0 to 17.9years) with SCD. A total of 100 childrenand adolescentswithSCD and 100 healthycontrols,similarinage, genderandethnicbackground,will bescreenedto documentthe prevalenceof vitaminBedeficiencyinchildrenwithSCD. FortychildrenwithserumPLP levels< 20 nmol/1,will be identifiedandrandomizedto receive either5 mg/day or25 rag/dayvitaminBefor 12 months. Forboth supplementationstudies,assessmentswill be madeat baseline,3, 6, 9 and 12 months.These two projectswillprovidesystematicinvestigationsof theroles of both vitaminA and vitaminBeinimprovinghealthoutcomes,growthand nutritionalstatus inchildrenwithSCD andwill potentiallyresultin cost-effectivenutritionalinterventionsthat can bequicklyimplementedintothe standardof care for thesechildren.
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