PEDSgNRg328gPedsgExamg2gCorrectgStudygGuide
HEMATOLOGICgDISORDERS: 7-10gquestions
• Identifyggeneralgnursinggstrategiesgassociatedgwithgalteredganemia
• Describegthegmorphology,gpathophysiology,gclinicalgmanifestations,gt
herapeuticgmanagementgandgnursinggconsiderationsgofgthegfollowingg
anemias:
o ***Laboratorygnorms:g6-12gyearsgofgage
▪ Hemoglobin:
• 11.5-15.5g g/dl
▪ Hematocrit:
• 35-45%
▪ MCVg(meangcorpusculargvolume)
• 77-95gfL
• MeasuregofgaveragegsizegofgRBC
• ↓g ing microcytic;g ↑g ing macrocytic
▪ MCHCg (meang corpuscularg Hgbg concentration)
• 31–37gg/dl
• ReflectiongofgHgbgconcentration
• Refergtogcolor;ginghypo-,gvaluesgareg↓
▪ MCHg(meangcorpusculargHgb)
• 25–33gpg/cell
• ReflectiongofgHgbgconcentration
• Refergtogcolor;ginghypo-,gvaluesgareg↓
o Anemia
▪ Conditiongwheregtheghemoglobingcontentgofgthegbloodgis
ginsufficientgtogsatisfygbodilygneeds
▪ Causes:
• Bloodgloss
• Acceleratedghemolysis
• Decreasedgproduction
▪ Typicalg Clinicalg Manifestations:
• Fatigue
• Dizziness
• Weakness
• Pallor
• ↑gheartgrate
• ↑gbreathinggrate
o Types:
▪ Iron-deficiencyganemia—MOSTgCOMMONgINgPEDS
• Nutritionalganemia
• Microcytic-hypochromic—small,gpalegRBC
• Who’sgatgrisk?
o Varied
o Prematuregbabiesgatg↑griskgb/cgtheygdon’tggetgthatg
bloodgrushgthegmomggivesgthegbabyglategingthatg3rdgt
rimester,gb/cgpremiesgaregborngearly—
thisgisgwhygprematureginfantsgneedgthegirongsupplem
entsgatg2-
3gmonths,gwhereasgtermgbabiesgdon’tgneedgitguntilg4
-6gmonths
, PEDSgNRg328gPedsgExamg2gCorrectgStudygGuide
o Multiplesgandgtwinsgatg↑gbecausegonlygfinitegamount
gofgbloodgavailablegingthatglastgrushgofgblood;gsognot
gallgthegbabiesggetgenoughgiron
o Breast-fedgexclusivelygbabiesgatg↑grisk—
breastgmilkgdoesn’tghavegsamegirongcontentgasgfort
ifiedgirongformula
o Kiddosgwithgchronicgbloodgloss—
GIgbleeders,ghemophiliacs
o Kiddosgwithgpoorgdietarygintake;gwholegmilkgdoesn’t
ghavegsamegirongconcentrationgasgirongfortifiedgfor
mula
• Pathophysiology:
o Bodygstoresgirongthatgisgusedgforgerythropoiesis
o Irongnecessarygmineralgforgerythropoiesisgtogoccur
o Notgenoughgirongtogtransportgtogthegbonegmarrowgto
gmakegRBCs
o BonegmarrowgstillgmakesgRBCsgbutgtheygaregverygs
mallgandghavegaglowerghemoglobingconcentration
• ClinicalgManifestations:
o Typicalgsignsgofganemiagplus:
▪ Irritability—duegtogtissueghypoxia/O2gcraving
▪ Picag(especiallygice,gdirt,gchalk,gpowdergorg
puregstarch)—
eatinggofgsubstancesgthatgaregnotgtypicalgof
gkiddosgtogeat;ghowevergthisgcouldgbeghardg
togdistinguishgfromgthisgoccurringgnormallyg
ingtoddlerhood
▪ Poorgmusclegtone
▪ Growthgretardation
▪ Headache
▪ Nailgbedgdeformitiesgcalled??gSpoongnail
▪ Tachycardic;gpossiblegmurmurs
• LabgValues:
o Everythinggisg↓↓↓↓gb/cgitsgmicrocytic-hypochromic
• Implementations:
o EDUCATION***
▪ Prevention*
▪ Highgirongfoods*
• Foodsgthatgagtoddlergwouldgeat;geggs,
gbreads
• Wholegmilkgtakesgupgroomgingbelly!!!gS
ogkiddogmaygnotgintakegenoughgiron-
grichgfoodgb/cgthey’regnotghungry;gre
membergwholegmilkgdoesgnotghavegsa
megirongconcentrationglikegfortifiedgfo
rmulagsogifgkiddogisgdrinkinggequalgam
ountsgofgwholegmilkgasghe/shegusedgto
gdrinkgthegformulaghe/shegisgnotggetti
nggenoughgiron
▪ Irong supplements
• Bestgongangemptygstomach
• BestgwithgVitamingC
,PEDSgNRg328gPedsgExamg2gCorrectgStudygGuide
• Don’tggivegwithgmilk
, PEDSgNRg328gPedsgExamg2gCorrectgStudygGuide
• Ifgtakinggefficientlygtheygwillghave
gblack/greengtarrygstools
• Labgvaluesgbestgindicator
• Usegagstrawgtogpreventgstaininggof
gteeth
• Monitorgforgconstipationg>reat
▪ Beta-Thallasemia
• Hemolyticganemia;gbonegmarrowgissuegisgthegunderlying
gpatho—
bloodgcellsgaregextremelygunstable,gRBCsglysegverygeasily
• Notgproducinggcorrectgbetagchainsgofghgb—
alphagandggammagattemptgtogtakegovergandgproducegRB
Csgthatgaregfragilegandgpronegtoglysing
• ***kiddosgBMgisgnotgmakinggrightghemoglobingch
ains—theygbreakgdowngverygquicklygandgeasily
• Microcytic-hypochromic
• Defectivegsynthesisgofgthegproteingcomponentgof
ghemoglobin
• Who’sgatgRisk
o Mediterraneang descent—Italiansg andg Greeks
• Pathophysiology:
o Hemoglobing synthesisg isg impaired
o Fragileg RBCg w/shortg life
o Severeganemia
• Types:
o Major
▪ Mostgsevere
▪ Occursginginfancy
▪ Ifgkiddogdoesn’tgreceivegfreq.gtransfusionsgan
dgtreatmentsgtheygrarelygsurvivegchildhood
▪ Withoutgtreatmentgtheygdiegbeforegageg7
o Intermediate
▪ Developgnormallygintogadulthood
▪ Needgtogbegtreated
▪ Delayedgpuberty
o Minor
▪ Carryg theg trait,g don’tg haveg it
▪ Havegnormalglife-span
• ClinicalgManifestations:
*goccurringgb/cgofgBMginvolvement
o Frequentgepistaxis*
o Osteoporosis*
o Pathologicgfractures*
o ChronicgCHF
HEMATOLOGICgDISORDERS: 7-10gquestions
• Identifyggeneralgnursinggstrategiesgassociatedgwithgalteredganemia
• Describegthegmorphology,gpathophysiology,gclinicalgmanifestations,gt
herapeuticgmanagementgandgnursinggconsiderationsgofgthegfollowingg
anemias:
o ***Laboratorygnorms:g6-12gyearsgofgage
▪ Hemoglobin:
• 11.5-15.5g g/dl
▪ Hematocrit:
• 35-45%
▪ MCVg(meangcorpusculargvolume)
• 77-95gfL
• MeasuregofgaveragegsizegofgRBC
• ↓g ing microcytic;g ↑g ing macrocytic
▪ MCHCg (meang corpuscularg Hgbg concentration)
• 31–37gg/dl
• ReflectiongofgHgbgconcentration
• Refergtogcolor;ginghypo-,gvaluesgareg↓
▪ MCHg(meangcorpusculargHgb)
• 25–33gpg/cell
• ReflectiongofgHgbgconcentration
• Refergtogcolor;ginghypo-,gvaluesgareg↓
o Anemia
▪ Conditiongwheregtheghemoglobingcontentgofgthegbloodgis
ginsufficientgtogsatisfygbodilygneeds
▪ Causes:
• Bloodgloss
• Acceleratedghemolysis
• Decreasedgproduction
▪ Typicalg Clinicalg Manifestations:
• Fatigue
• Dizziness
• Weakness
• Pallor
• ↑gheartgrate
• ↑gbreathinggrate
o Types:
▪ Iron-deficiencyganemia—MOSTgCOMMONgINgPEDS
• Nutritionalganemia
• Microcytic-hypochromic—small,gpalegRBC
• Who’sgatgrisk?
o Varied
o Prematuregbabiesgatg↑griskgb/cgtheygdon’tggetgthatg
bloodgrushgthegmomggivesgthegbabyglategingthatg3rdgt
rimester,gb/cgpremiesgaregborngearly—
thisgisgwhygprematureginfantsgneedgthegirongsupplem
entsgatg2-
3gmonths,gwhereasgtermgbabiesgdon’tgneedgitguntilg4
-6gmonths
, PEDSgNRg328gPedsgExamg2gCorrectgStudygGuide
o Multiplesgandgtwinsgatg↑gbecausegonlygfinitegamount
gofgbloodgavailablegingthatglastgrushgofgblood;gsognot
gallgthegbabiesggetgenoughgiron
o Breast-fedgexclusivelygbabiesgatg↑grisk—
breastgmilkgdoesn’tghavegsamegirongcontentgasgfort
ifiedgirongformula
o Kiddosgwithgchronicgbloodgloss—
GIgbleeders,ghemophiliacs
o Kiddosgwithgpoorgdietarygintake;gwholegmilkgdoesn’t
ghavegsamegirongconcentrationgasgirongfortifiedgfor
mula
• Pathophysiology:
o Bodygstoresgirongthatgisgusedgforgerythropoiesis
o Irongnecessarygmineralgforgerythropoiesisgtogoccur
o Notgenoughgirongtogtransportgtogthegbonegmarrowgto
gmakegRBCs
o BonegmarrowgstillgmakesgRBCsgbutgtheygaregverygs
mallgandghavegaglowerghemoglobingconcentration
• ClinicalgManifestations:
o Typicalgsignsgofganemiagplus:
▪ Irritability—duegtogtissueghypoxia/O2gcraving
▪ Picag(especiallygice,gdirt,gchalk,gpowdergorg
puregstarch)—
eatinggofgsubstancesgthatgaregnotgtypicalgof
gkiddosgtogeat;ghowevergthisgcouldgbeghardg
togdistinguishgfromgthisgoccurringgnormallyg
ingtoddlerhood
▪ Poorgmusclegtone
▪ Growthgretardation
▪ Headache
▪ Nailgbedgdeformitiesgcalled??gSpoongnail
▪ Tachycardic;gpossiblegmurmurs
• LabgValues:
o Everythinggisg↓↓↓↓gb/cgitsgmicrocytic-hypochromic
• Implementations:
o EDUCATION***
▪ Prevention*
▪ Highgirongfoods*
• Foodsgthatgagtoddlergwouldgeat;geggs,
gbreads
• Wholegmilkgtakesgupgroomgingbelly!!!gS
ogkiddogmaygnotgintakegenoughgiron-
grichgfoodgb/cgthey’regnotghungry;gre
membergwholegmilkgdoesgnotghavegsa
megirongconcentrationglikegfortifiedgfo
rmulagsogifgkiddogisgdrinkinggequalgam
ountsgofgwholegmilkgasghe/shegusedgto
gdrinkgthegformulaghe/shegisgnotggetti
nggenoughgiron
▪ Irong supplements
• Bestgongangemptygstomach
• BestgwithgVitamingC
,PEDSgNRg328gPedsgExamg2gCorrectgStudygGuide
• Don’tggivegwithgmilk
, PEDSgNRg328gPedsgExamg2gCorrectgStudygGuide
• Ifgtakinggefficientlygtheygwillghave
gblack/greengtarrygstools
• Labgvaluesgbestgindicator
• Usegagstrawgtogpreventgstaininggof
gteeth
• Monitorgforgconstipationg>reat
▪ Beta-Thallasemia
• Hemolyticganemia;gbonegmarrowgissuegisgthegunderlying
gpatho—
bloodgcellsgaregextremelygunstable,gRBCsglysegverygeasily
• Notgproducinggcorrectgbetagchainsgofghgb—
alphagandggammagattemptgtogtakegovergandgproducegRB
Csgthatgaregfragilegandgpronegtoglysing
• ***kiddosgBMgisgnotgmakinggrightghemoglobingch
ains—theygbreakgdowngverygquicklygandgeasily
• Microcytic-hypochromic
• Defectivegsynthesisgofgthegproteingcomponentgof
ghemoglobin
• Who’sgatgRisk
o Mediterraneang descent—Italiansg andg Greeks
• Pathophysiology:
o Hemoglobing synthesisg isg impaired
o Fragileg RBCg w/shortg life
o Severeganemia
• Types:
o Major
▪ Mostgsevere
▪ Occursginginfancy
▪ Ifgkiddogdoesn’tgreceivegfreq.gtransfusionsgan
dgtreatmentsgtheygrarelygsurvivegchildhood
▪ Withoutgtreatmentgtheygdiegbeforegageg7
o Intermediate
▪ Developgnormallygintogadulthood
▪ Needgtogbegtreated
▪ Delayedgpuberty
o Minor
▪ Carryg theg trait,g don’tg haveg it
▪ Havegnormalglife-span
• ClinicalgManifestations:
*goccurringgb/cgofgBMginvolvement
o Frequentgepistaxis*
o Osteoporosis*
o Pathologicgfractures*
o ChronicgCHF
