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Anemia of Inadequate
production
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Anaemia
Hemoglobin level below the normalrange for the age and sex
• Neonate < 14 gr/dl
• 11! months < 1" gr/dl
• 11! #ears < 11 gr/dl
• Hemoglobin at birth $ cord blood % 1&1' gr/dl• !44' hour of age $ Increase due to hemoconcentration• ( months of age )rop up to *+, $ 11 gr/dl
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-auses of AnaemiaImpaired red cell production
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Approach to -ommon -auses of Anemia in -hildren
)#smorphic
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Iron )ecienc# Anemia
• Iron .etabolism
• 0tiolog#
•
-linical manifestations• aborator# investigations
• )i2erential )iagnosis
•
3reatment
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Iron .etabolism
• 3otal bod# iron content – New born bab# $ !," mg nelson "+, gr 5
– Adult 4 , gr
• Iron requirement – 3o fulll the decit 4+, gr over 1, #ears
• 4+, gr/(&, % "+' mg/da#
– 3o replace Iron loss via epithelial shedding
– 3otal elemental iron requirement % 1 mg/6g/d
Iron requirement• 7ne #ear old infant % 'mg/da#• 8ather % * mg/da#
• .other %1, mg/da#
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Iron inta6e
• 9reast mil6 $ 1+, mg/ $ ,": absorbed
• Infant formula $ ,* ;1!5 mg/ $ 1": absorbed
– reterm formula 1,mg/l for b+wt < 1'"" gr
•
-ow=s mil6 $ "+, mg/ $ 1": absorbed• .ixed diet $ 4* mg/da# 1"1,: absorbed
– Interfered b# food $ tea tanin5> -aws mil6 calcium5>High ber foods ph#tate5• Iron from cereals onl# 1: is absorbed
– 8acilitated b# $ 9reast mil6> vitamin -
– Iron supplementation• After 4 months for exclusivel# breast fed babies
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Iron )ecienc# and Anemia
• ?@ – 1! #ears $ *: decient ( : anemic
nelson5
–
Adolescent *: iron decient
!:anemicnelson5
– revalence of iron decienc# 1': whites and ((:immigrants illustrated5
•
@ri an6a – ("4" : anemic
– Iron )ecienc#
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0tiolog#
• Inadequate inta6e iron reclaimed b# drop in Hb in !(mo5 – 0arl# 9B or unusual blood loss at birth
– *!4 mo 3erm babies
• Absorption – Interfered b# cows mil6>
– 8acilitated b# Cit -
• 9lood loss – DI3 $ pol#p> hemangioma> inEammator# bowel disease>
Hoo6worm> -hronic diarrhea> cows mil6 protein allerg# – 9lood loss due to heat labile protein in cows mil6
– 9lunting of villi due to iron decienc# can lead to loss of iron
– Intense exercise conditioning in girls
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-linical .anifestations
• allor
• Irritabilit# F behavioral problems
•
Impaired neurological andintellectual functions girls with @erum ferritin level lessthan 1! ng/l improved after ' wee6s of iron5
• agophagia
• -ardiac $ tach#cardia> cardiomegal#> s#stolicmurmur
• 9reath holding attac6s are reduced b# irontherap#Iron is found in .onoamine ox#dase> c#tochromes > catalase and peroxidase
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aborator# 8indings in iron
decienc#
• @erum ferritin
• @erum iron
• Iron binding capacit#
• 3ransferin saturation
• 8ree 0r#throc#te protoporph#rine 085
• .-C F .-H .icroc#tic> H#po chromic5
• 9lood picture $ hoi6iloc#tosis> NucleatedG9-
• Geticuloc#te relative 5
•
3hromboc#tosis similarit# of er#thropoietin F 3hrombopoitin5
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)eferential )iagnosisH#po -hromic .icroc#tic Anemia
• 3halasaemia trait beta> alpha> 05 – G9- count raised in thal trait> not in iron decienc#
– G)B is wide in iron decienc#
• Anemic of chronic disease usuall# Normoc#tic but can be .icroc#tic5
• ead poisoning• 7ther .icroc#tic anemia
– @ideroblastic anemia dimorphic and high G)B> serum iron increased>transferring saturation increased and ring sideroblasts in the marrow5
• -ongenital sideroblastic anemia lin6ed withlioniation5
– AtransferinemiaJ congenital absence of iron bindingprotein treated with apotransferrin5
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3reatment
• Gegular response to ironK
• @imple iron salts can be used sulphate>gluconate>fumarate5
–
@odium iron editate and pol#sacharide ironcomplex does not stain teeth
• )ose 4& mg/6g of elemental iron in threedoses
– onl# !": of ferrous sulphate is elemental iron• )uration
– ( months
– ' wee6s after blood value come to normal
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3reatment ct+
• Intolerance to iron is uncommon in #oungchildren than adolacents and adult
• -onsumption of mil6 should be reduced
• 0xpected rate of Hb rise 1 gr/dl/wee6 "+,mg/dl/da#5
• 9lood transfusion not necessar# and canbe dangerous due to h#pervolemia and
cardiac dilatation+ – Hb value less than 4 gr/dl should get onl#
4ml/6g of pac6ed cells at a time+
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Gesponse to iron therap# in iron
decienc# anemia
Time afterironadministra
tion
Response
1!%!4hours
Geplacement of intracellulariron> subLective improvementJdecreased irritabilit#>
increased appetite
(&4' hours Initial bone marrow response Jer#throid h#perplasia
4';! hours Geticuloc#tosis> pea6ing at ,;
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.egaloblastic Anemias
1+ Ine2ective er#thropoiesis $ Activeer#thropoiesis with premature death of cells
!+ High .-C and h#per segmented B9-(+ )ecienc# of 91! or folate and rare
metabolic disorders
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8olate decienc#
• 8olate abundant in green vegetableand animal food M heatlabile pol#glutamate5
• ol#glutamate monoglutamate b#folate conLugase in entire intestinalbrush border
• 8olic acid 3etrah#drofolate biological# active form5 b# 3etraH#dro 8olate reductatse en#me
• 9od# stores are limited $ Anemia in
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-linical features
• Neonate and infant – -ommon in Cer# low birth weight babies $
routine supplementation
–
ea6 incidence $ 4; mo earlier than irondecienc#5
– @igns and s#mptoms related to anemia
– Irritable> fail to gain weight> chronic diarrhea
–
3hromboc#topenia and Hemorrhage• 7lder children
– @igns and s#mptoms related to anemia
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0tiolog#
• Inadequate inta6e
• )ecreased absorption
•
-ongenital abnormalities in folatemetabolism
• )rug induce abnormalities of folatemetabolism
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0tiolog#
• Inadequate inta6e – Increased demand pregnanc#> infanc#> hemol#sis5 – Aggravate b# infections
• )ecreased absorption
– -hronic diarrhea and other bowel disease
– Antiepileptic drugs hen#toin> primidone>henobarbital5
• -ongenital abnormalities in folate metabolism
– 3reated with high doses of folate
• )rug induce abnormalities of folate metabolism – .3> 3rimethoprim> #rimethmine
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aborator# ndings in 8olic acid
)ecienc#
• .acroc#tic $ .-C 1"" E
• Geticuloc#te count low
•
Neucleated G9-• Neutropenia and thromboc#topenia
• Geduced G9- folate level normal 1,"
&"" ng/ml5• 9one marrow h#percelluler
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3reatment
• 8olic acid $ "+, 1 mg/da#
• High doses ma# correct 91!decienc# anemia also+ 3herefore Ifthe diagnosis is in doubt J trial of folicacid "+1 mg/da# for one wee6 andsee the response
• )uration of treatment $ (4 wee6
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Citamin 9 1! )ecienc#
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Citamin 91! -obalamin5 decienc#
• -obalamin produced b# microorganisms
• @tomach $ -ombine with G protein and I8
• )uodenum $ 9rea6 down G protein b# pancreatic en#mes
• Ileum distal5 $ absorption
• lasma $ 9ind to trans -obalamin II andtransported to liver> bone marrow>
•
-ells $ Geceptor mediated endoc#tosis and converted tometh#lcobalomin and adenos#l cobolamin J 3ransfer of meth#lgroups to )NA
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0tiolog# of 91! )ecienc#
• Inadequate Inta6e
• ac6 of intrinsic factor
•
Impaired Citamin b1! absorption• Absence of Citamin 91! transport
protien
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0tiolog# of 91! )ecienc#
• Inadequate Inta6e
– @tores suOcient for (4 #ears neonates 4, mo5
– @trict vegetarians and their babies
• ac6 of intrinsic factor
– -ongenital ernicious anemia – Puvenile pernicious anemia
– Dastric surger#
• Impaired Citamin b1! absorption
–
Intestinal pathologies or resections N0-> I9)5 – 9acterial overgrowth or tapeworm
• Absence of Citamin 91! transport protein
– 3- II decienc# normal 91! level due to 3-I and 3-III5
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-linical features of 91!)ecienc#
• Deneral $ Bea6ness> fatigue> 8ailureto thrive> irritabilit#> pallor> glossitis>Comiting> diarrhea> icturus+
• Neurologic $ aresthesia> sensor#decit> H#potonia> seiures>development dela#> regression>
neurops#chiatric changes• H#poreEexia babins6i responses and
clonus
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aborator# ndings in 91!
)ecienc#
• Identical with folate decienc#
• @erum 91! levels below 1"" pg/ml
• Increased levels of meth#lmelonic acid in urine
normal " (+, mg/!4 hr5• @chilling test
– -,; labeled 9 1! 7rall#
– High dose Eushing dose5 of 91! aretaral ! hrs later
–
Normal $ 1"(": -,;
labeled 9 1! excretion in urine – I8 decienc# $
– Intestinal patholog# $ Not corrected b# I8
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3reatment of 91! decienc#
• arenteral 91! 1 mg5
• Gesponse in !4 da#s
•
Neurological deciencies I. dosesdail# for ! wee6s
• .aintenance $ .onthl# 1 mg I. forlife
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7ther .egaloblastic Anemia
• 7roticaciduria
• 3hiamineGesponsive .egaloblasticAnemia
• )ecienc# of adenos#l -obalaminand meth#l -obalamin
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h#siologic Anemia ofInfanc#
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h#siologic Anemia ofInfancy
• At birth Hb $ 1'!" gr/dl
• At '1! w6 Hb *11gr/dl $ h#siologic anemia5
• Geasons for ph#siologic anemia
–
Increase of availabilit# of ox#gen saturation fromfetus to new born from ,": to *,:
– -hange the t#pe of Hb Hb8 HbA5
– )own regulation of erthropoitin production
–
0r#thropoiesis is suppressed• Iron is rec#cled and suOcient up to !" w6s
with no inta6e
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h#siologic Anemia of Premature Infants
• .ore rapid and extreme drop of Hb ;*gr/dl b# (& wee6s ma# be lower in Cer# prematurebabies5
• -auses for anemia of premature infants
–
9lood loss due to @ampling – @uboptimal er#thropoietin response
hepatic Genal occur around 4"wee6s 5
– @hort survival of G9- 4"&" da#s Cs 1!"da#s
– Gapid growth
–
@low ongoing hemol#sis
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h#siologic Anemia of Premature Infants
• 3reatment $ on the clinical condition can tolerate up to &+,gr/dl5
• 8olic acid and iron 4& mg/6g/d5
• 9lood transfusion $ 1"1, ml/6g
• 0r#thropoietin
• revention – .inimie blood loss b# sampling
, ml from a '"" gr bab# % ,"" ml from a '" 6g '"
""" gr5 man
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-auses of AnaemiaImpaired red cell production
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• )iamond9lac6fan Anemia
• 3ransient 0r#throblastopenia of
childhood 30-5
• arvovirus 91* infection
• Anemia of -hronic )isorder A-)5 F
AG8
• -ongenital )iser#thropoietic Anemia-)A5
her -auses Anemia of inadequate producti
d cell aplasia
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)enition for @evere Aplastic
Anaemia
• 3wo or more of the following cell lineare compromised in a patient withmoderate or severel# h#po cellular
bone marrow
– Absolute neutrophil count less than
,""/mm(
– A latelet count less than !" """/mm(
– A reticuloc#te count less than 1: after
correction for the hematocrit
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Aplastic Anaemia
• Geduction of all three cell lines $ anc#topenia – G9- $ Anaemia
– latelets $ 3hromboc#topenia
– Bhite cells $ leucopenia
• -auses of Aplastic anaemia – Inherited
• Infections $ hepatitis
• )rugs $ @ulphonamides> chemotheraph#
• 3oxins $ 9enene> glue
– Acquired $ 8anconi anaemia
– Idiopathic
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8anconi Anemia
8 i A i -li i l
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8anconi Anaemia $ -linicalfeatures
• .ost comment inherited anaemia
• Autosomal recessive inheritance
• Associated with congenital abnormalities – @hort stature
– Abnormal radii and thumb
– Genal malformations
–
.icropthalmia – igmented s6in lesions
• @igns of bone marrow failure usuall# appears at ,& #rs ofage
8 i i
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8anconi anaemia $)iagnosis
• Neonates with 8anconi nearl# alwa#shas normal blood counts
• Increased chromosomal brea6age ofperipheral blood cells
• )eath due to aplastic anaemia orleu6emia
• 3reatment $ 9one marrow transplantfrom una2ected siblings
@ h )i d
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@chwaman )iamonds#ndrome
• Gare
• Autosomal recessive
• -linical features – Aplastic anaemia
– ancreatic exocrine failure
– @6eletal abnormalities
• Gis6 of acute leu6emia
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3ransient 0r#throblastopenia of -hild
hood 30-
• 3he most common acquired red cellaplasia
• -ommoner than congenital red cell
aplasia
• & months to ( #ears of age mostabove 1 #r5
• 3ransient immunologic suppressionof er#thropoiesis ma# follow viralinfections but not arvovirus 91*
• Neutropenia in !":
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3ransient 0r#throblastopenia of -hild
hood 30-
• .-C $ normal
• Hb8 $ Not present
• Ged -ell A)A levels $ Normal highin other congenital aplastic Anemias5
• Gecover within 1! months
• 3ransfusions ma# be required usuall#onl# one5
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Acquired anc#topenias
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Acquired anc#topenias
• Gadiation
• Immune diseases
• regnanc#• arox#smal Nocturnal
Hemoglobinuria
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