of 84
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The Child with Hematologicor Immunologic Dysfunction
Chapter 26
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Assessment of
Hematologic Function Complete blood count History and assessment ndings
Childs energy and acti!ity le!el
"rowth patterns
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Anemia
The most common hematologic disorder ofchildhood
Decrease in number of #$Cs and%or hemoglobinconcentration below normal
Decreased o&ygen'carrying capacity of blood
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Classication of Anemias
(tiology and physiology
#$C and%or Hgb depletion
)orphology
Characteristic changes in #$C si*e+ shape+ and%or
color
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Conse,uences of Anemia
Decrease in o&ygen'carrying capacity of bloodand decreased amount of o&ygen a!ailable totissues
-hen anemia de!elops slowly+ child adapts
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(.ects of Anemiaon Circulatory /ystem Hemodilution Decreased peripheral resistance Increased cardiac circulation and turbulence
)ay ha!e murmur
)ay lead to cardiac failure Cyanosis "rowth retardation
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Diagnostic (!aluation
C$C
Decreased #$Cs
Decreased Hbg and Hct
0ther tests for particular type of anemia
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Therapeutic
)anagement Treat underlying cause Transfusion after hemorrhage if
needed
Nutritional intervention for deciencyanemias
Supportive care
IV uids to replace intravascular
volume Oxygen
Bed rest
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1ursing Considerations
repare child and family for laboratory tests
Decrease o&ygen demands
re!ent complications
/upport family
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Iron Deciency Anemia
Caused by inade,uate supply of dietary iron
"enerally pre!entable
Iron'fortied cereals and formulas for infants
/pecial needs of premature infants
Adolescents at ris3 due to rapid growth and pooreating habits
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Iron Deciency Anemia
Most common nutritional prolem in !S "aused y any numer of factors that
decrease the supply of #e$ impair itsasorption$ increase the ody%s needfor #e$ or a&ect the synthesis ofhemogloin'
(arge mil) inta)e *ith lo* solid foodsource
+apid gro*th
Inade,uate diet$ menses$ impairedasorption
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Iron Deciency Anemia
athophysiology facts Birth- full term infant
#e supply./00mg
-1uring the last
trimester-#e transferfrom mom 2 rateof 3mg4day
Maternal #e storesare ade,uate for upto 3-5 months
If diet doesn%tsupplement thematernal4fetalstores
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Iron Deciency AnemiaClinical )anifestations
Mil) ay-over*eight Mil) is a poor source of iron$
Vit'"$ 6inc 7 #louride
8ale$ porcelain-li)e s)in !nder*eight 8oor muscle dev$edema$
retarded gro*th$ Irritale$ tachycardic$ fatigue$
)oilonychia Social and cognitive ailities
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Iron Deciency Anemia
re!ention%/creening%Teaching Dietary counseling Iron Fortied formula%cereal up to 42 m Iron rich foods upon solids Fe supplementation 5Ferrous sulfate 'gi!e b%w meals%7it C to absorption
'8eep no more than 4m supply9to&ic
Mgmt4Nursing
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/ic3le Cell Anemia
A hereditary hemoglobinopathy
(thnicity
0ccurs primarily in blac3s
0ccurrence 4 in :;< infants born in =/ 4 in 42 ha!e sic3le cell trait
0ccasionally also in persons of)editerranean descent
Also seen in /outh American+ Arabian+and (ast Indian descent
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(tiology of /ic3le Cell
In areas of world where malaria is common+indi!iduals with sic3le cell trait tend to ha!esur!i!al ad!antage o!er those without trait
Autosomal recessi!e disorder
4 in 42 blac3s are carriers 5ha!e sic3le celltrait
5If both parents ha!e trait+ each o.springwill ha!e 4 in > li3elihood of ha!ing disease
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athophysiology
artial or complete replacement of normal Hgbwith abnormal hemoglobin / 5Hgb /
Hemoglobin in the #$Cs ta3es on an elongated?sic3le@ shape
/ic3led cells are rigid and obstruct capillary bloodow
)icroscopic obstructions lead to engorgement andtissue ischemia
Hypo&ia occurs and causes sic3ling
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/ic3led Hemoglobin
=nder conditions of dehydration+ acidosis+hypo&ia+ and temperature ele!ations+ Hgb/
changes its structure in the cell membrane froma pliable dis3 to a crescent or sic3le shaped #$CB
/ic3ling response is re!ersible with o&ygenation hydration
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athophysiology
arge tissue infarctions occur
Damaged tissues in organsE impaired function
/plenic se,uestration
)ay re,uire splenectomy at early age
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rognosis
No cure 9except possily one marro*transplants:
Supportive care4prevent sic)lingepisodes
#re,uent acterial infections may occurdue to immunocompromise Bacterial infection is leading cause of
death in young children *ith sic)le celldisease
Stro)es in ;
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/ystems A.ected
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/ic3le Cell Crisis
recipitating factors Anything that increases bodys need for o&ygen or
alters transport of o&ygen Trauma Infection+ fe!er
hysical and emotional stress Increased blood !iscosity due to dehydration Hypo&ia
From high altitude+ poorly pressuri*ed airplanes+hypo!entilation+!asoconstriction due tohypothermia
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/ic3le Cell Crisis 5contd
Acute e&acerbations that !ary in se!erity andfre,uency
Types
7aso'occlusi!e 70CG thrombotic )ost common type of crisis!ery painful
/tasis of blood with clumping of cells inmicrocirculationischemiainfarction
/ignsJ fe!er+ pain+ tissue engorgement
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/ic3le Cell Crisis 5contd
Types 5contd
/plenic se,uestration
ife threateningdeath can occur within hours
$lood pools in the spleen
/igns rofound anemia+ hypo!olemia+ and shoc3
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Diagnosis of /ic3le Cell
Cord blood in newborns
1ewborn screening done in >: states
"enetic testing to identify carriers and childrenwho ha!e disease
/ic3le'turbidity test
Kuic3 screening purposes in children L6 mos of age
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)edical )anagement
Aggressi!e treatment of infection
ossible prophylactic antibiotics from 2 mos'< yrs
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)edical )anagement
5contd )onitor reticulocyte count regularly to e!aluatebone marrow function
$lood transfusion+ if gi!en early in crisis+ mayreduce ischemia
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)edical )anagement
5contd Fre,uent transfusion decreases hemosiderosis5iron in tissues
Treat with iron'chelation such as fero&amine M!itamin C to promote iron e&cretion
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1ursing )anagement
)onitor childs growthwatch for failure to thri!e
Careful multi'system assessment
Assess pain
0bser!e for presence of inammation or possibleinfection
Carefully monitor for signs of shoc3
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sychosocial 1eeds
Coping mechanisms
/upport with genetic counseling
Financial needs
Caregi!er role strain
i!ing with chronic illness in the family
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Hemophilia
A group of hereditary bleeding disorders thatresult from deciencies of specic clotting factors
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Hemophilia
N lin3ed recessi!e disorder
Carried on female chromosomeE males are
a.ected Deciency of Factor O+ produced by the
li!er+ necessary for the formation ofthromboplastin for clotting
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Types of Hemophilia
Hemophilia A
?Classic hemophilia@
Deciency of factor 7III
Accounts for OPQ of cases of hemophilia
0ccurrenceJ 4 in
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Hemophilia A'/e!erity
Degrees /e!ere'/pontaneously bleed w%o trauma )oderate'$leed with trauma
)ild'se!ere trauma%surgery
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Types of Hemophilia
5contd Hemophilia $ Also 3nown as Christmas disease
Caused by deciency of factor IN
Accounts for 4
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)anifestations of
Hemophilia $leeding tendencies range from mild tose!ere
/ymptoms may not occur until 6 mos of age
)obility leads to inRuries from falls andaccidents
Hemarthrosis
$leeding into Roint spaces of 3nee+ an3le+
elbow leading to impaired mobility (cchymosis
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)anifestations 5contd
(pista&is
$leeding after procedures
)inor trauma+ tooth e&traction+ minor surgeries
arge subcutaneous and intramuscular hemorrhages
may occur $leeding into nec3+ chest+ mouth may compromise
airway
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Clinical Therapy
Can be diagnosed through amniocentesis
"enetic testing of family members to identifycarriers
Diagnosis on basis of h&+ labs+ and e&am
SabsJ ow le!els of factor 7III or IN+ prolonged TT
S1ormalJ platelet count+ T+ and brinogen
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)edical )anagement
11>V8 IV "auses ?-3 @ increase in factor
VIII activity !sed for mild hemophilia
+eplace missing clotting factors Transfusions
>t home *ith prompt interventionto reduce complications
#ollo*ing maAor or minorhemorrhages
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rognosis
Historically+ most died by < yrs age
1ow mild to moderate hemophilia patients li!enear normal li!es
"ene therapy for future
Infused carrier organisms act on target cells topromote manufacture of decient clotting factor
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Inter!entions
"lose supervision and safeenvironment
1ental procedures in controlled
situation
Shave only *ith electric ra6or
Supercial leedingapplypressure for at least =; minutesC ice to vasoconstrict
If signicant leeding occurs$transfuse for factor replacement
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)anaging Hemarthrosis
During bleeding episodes+ ele!ate and immobili*eRoint
Ice Analgesics #0) after bleeding stops to pre!ent contractures T A!oid obesity to minimi*e Roint stress
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7on -illebrand Disease
> hereditary leeding disorder$ involvingdeciency of Von Dillerand factor$ 9aplasma protein$ and the carrier forfactor VIII:
Von Dillerand factor is necessary forplatelet adhesion
Transmitted as autosomal dominant trait
Occurs in males and females
Eene for disease is located onchromosome =?
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)anifestations of7on -illebrand Disease (asy bruising
(pista&is
"ingi!al bleeding
(&cessi!e bleeding with lacerations or surgeries
)enorrhagia
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Diagnosisaboratory Findings Decreased 7on -illebrand factor le!els
7on -illebrand antigen le!els
Decreased platelet agglutination
rolonged bleeding time
TT may be normal or prolonged
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Treatment of7on -illebrand Disease Infusion of 7on -illebrands protein concentrate
DDA7 infusion prior to surgery or to treatbleeding episode 5synthetic !asopressin
Aminocaproic acid 5Amicar to treat bleeding in
mucous membranes 5in some cases
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Inter!entions
A!oid aspirin or 1/AIDs 5increased bleeding timeand inhibit platelet function
)anage bleeding episodes with prompt infusiontherapy
Children with 7on -illebrands ha!e normal lifee&pectancy if well managed
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IdiopathicThrombocytopenic urpura5IT An ac,uired hemorrhagic disorder characteri*ed
by
ThrombocytopeniaJ e&cessi!e destruction ofplatelets
urpuraJ discoloration caused by petechiae beneaththe s3in
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IT Forms
Acute self'limiting
0ften follows =#I or other infection
Chronic 5L6 months duration
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IT
Diagnostic e!aluation
Therapeutic management
rognosis
1ursing considerations
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(pista&is 51osebleeding
Isolated and transient epista&is is common inchildhood
#ecurrent or se!ere episodes may indicateunderlying disease
7ascular abnormalities+ leu3emia+ thrombocytopenia+clotting factor deciency diseases 57on -illebranddisease and hemophilia
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1ursing Considerations(pista&is #emain calm+ 3eep child calm
Ha!e child sit up and lean forward
ressure to nose
Further e!aluation if bleeding continues
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1eoplastic Disorders
eading cause of death from disease in childrenpast infancy
Almost half of all childhood cancers in!ol!e bloodor blood'forming organs
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eu3emias
)ost common form of childhood cancer
:'> cases per 4PP+PPP caucasian children 4< yrsold
)ore fre,uent in males L4 yr old
ea3 onset between 2 and 6 yrs of age
/ur!i!ability
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Classication of
eu3emias eu3emiaJ A broad group of malignant disease ofbone marrow and lymphatic system Comple& disease with !arying heterogeneity
Classications are increasingly comple&
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)orphology
Acute lymphoid leu3emia 5A
Acute non'lymphoid 5myelogenous leu3emia5A1 or A)
/tem cell or blast cell leu3emia
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/ymptoms
AJ lymphatic+ lymphocytic+ lymphoblastic+ andlymphoblastoid leu3emia
A)J granulocytic+ myelocytic+ monocytic+myelogenous+ monoblastic+ and non'myeloblastic
leu3emia
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rognosis
A'SSinitial -$C countSS5
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athophysiology
eu3emia is an unrestricted proliferation ofimmature -$Cs in the blood'forming tissues ofthe body
i!er and spleen are the most se!erely a.ected
organs
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athophysiology 5contd
Although leu3emia is an o!erproduction of -$Cs+often acute form causes low leu3ocyte count
Cellular destruction ta3es place by inltration andsubse,uent competition for metabolic elements
C f
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Conse,uences of
eu3emia Anemia from decreased #$Cs Infection from neutropenia
$leeding tendencies from decreased plateletproduction
/pleen+ li!er+ and lymph glands show mar3edinltration+ enlargement+ and brosis
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Diagnostic (!aluation
$ased on history+ physical manifestations
eripheral blood smear
Immature leu3ocytes
Fre,uently low blood counts
to e!aluate C1/ in!ol!ement $one marrow aspiration or biopsy
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)gmtB%Follow rotocols
Chemotherapeutic agents
Cranial irradiation 5in some cases
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)gmtB%Follow rotocols
"hemotherapy9antineoplasticagents:
+adiotherapy Immunotherapy
3 phasesF Induction Intensication4co
nsol' "NS prophylax Maintenance
BMTFallogeneic4autologous
Prepare families for dx/txprocedures
Provide cont emotionalsupport
Reverse isolation FYI=BMA center of the
one is hollo! andcontains the cells that laterecome !or"in#$ loodcells or leu"emic cells
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Four hases of Therapy
Induction therapyJ >'6 wee3s
C1/ prophylactic therapyJ intrathecalchemotherapy
Intensication 5consolidation therapyJ To
eradicate residual leu3emic cells and pre!entresistant leu3emic clones
)aintenance therapyJ to preser!e remission
H t i ti /t C ll
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Hematopoietic /tem CellTransplantation 5H/CT
Donors may be relati!es or non'relati!es
Antigen'matched or mismatched
eripheral stem cells may be used
/tem cells from umbilical cord blood
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#is3s of H/CT
/ignicant ris3 of morbidity and mortality
"raft !sB host disease 5"7HD
0!erwhelming infection
/e!ere organ damage
Cure after H/CTJ up to 6PQ';PQ
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rognosis
If relapse after H/CTJ dismal prognosis Identied factors for determining prognosis
Initial -$C count Age at time of diagnosis Type of cell in!ol!ed
"ender 8aryotype analysis
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1ursing Considerations
Assessment
1ursing diagnosis
lanning
Implementation
repare child and family for procedures
ain management
re!ent complication of myelosuppression
Increased /usceptibility
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Increased /usceptibilityto Infection At time of diagnosis and relapse
During immunosuppressi!e therapy
After prolonged antibiotic therapy thatpredisposes to the growth of resistant organism
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Infection Control
(n!ironment
Hand hygiene
7isitor restriction
Nursing "are
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Infection Control
Manage prolemsof irradiation anddrug toxicity .
N4V$ anorexia$mucosalulceration$neuropathy$hemorrhagic
cystitis$ alopecia$moon face$ moodchanges$ pain
Nursing "are
provide favored foods
mouth care, soft toothbrush,mouthwash, hydrogen
peroxide, saline
Vincristine, numbness temp
temporary, wigs, hats
decrease Na intake, look
healthyanalgesics
)anaging
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)anagingChemotherapeutic Agents ?7esicants@sclerosing agents e!en in minute
amounts
Inter!entions for e&tra!asation
#is3 for anaphyla&is
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1ursing Diagnoses
#is3 for inRury related to malignant process+treatment
#is3 for uid !olume decit related to nausea+!omiting
Altered nutrition
Impaired s3in integrity
Altered family processes
Fear related to diagnosis+ procedures+treatments
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ymphomas
Hodg3in disease )ore pre!alent in 4 yrs of age
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Hodg3in Disease
1eoplastic disease originating in lymphoid system
0ften metastasi*es to spleen+ li!er+ bone marrow+lungs+ and other tissues
i i
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Diagnostics
Clinical manifestations of Hodg3in disease
ymph node biopsy for diagnosis and staging
resence of #eed'/ternberg cells is characteristicof Hodg3in disease
Therapeutic
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Therapeutic)anagement #adiation
Chemotherapy 5alone or with radiation
rognosis
1ursing considerations
1 H d 3i h
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1on'Hodg3in ymphoma
Appro&imately 6PQ of pediatric lymphomasare 1H
Clinical appearance Disease usually di.use rather than nodular
Cell type undi.erentiated or poorlydi.erentiated Dissemination occurs early+ often+ and
rapidly )ediastinal in!ol!ement and in!asion of
meninges
Immunologic Deciency
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Immunologic DeciencyDisorders
Severe Combined ImmunodeficiencySyndrome (SCID) and Wiskott-AldrichSyndrome-the body is unable to mount an
immune response.he immune system!s function is toreco"ni#e $self% from $nonself% and toinitiate a response to eliminate the
$nonself% or anti"en.Cell surface markers&body cells 'ithspecific cell surface markers uniue to theindividual
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rotecti!e )echanisms
Intact s)in Saliva$ s*eat$
tears$ stomachacids
Snee6ing$coughing
8rimary lymphoidorgans9thymus$BM$liver:
Secondarylymphoid organs9spleen$ lymphnodes$ E>(T:
7ertical Transmission
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7ertical Transmission5erinatal
HI7 M mother to her newborn
Antepartum
IntrapartumJU deli!ery ostpartumJ !ia breastfeeding'minimal in =B/B+
carries a ris3 as high as 2OQ
1 i # ibiliti
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1ursing #esponsibilities
GIt%s the (a*H
M%&' counsel ( offer testin# to those !hoappear at deliver) !ith *+ record of an ,I- testdurin# pre#nanc)
If a !oman declines ,I- testin#. a si#nedoection M%&' e attempted
+ffer testin# to a child. !hose mother has not
een tested 0hilds ,I- status is strictl) confidential. schools
ma) not have info unless parents allo!
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Therapeutic Mgmt'
Assess compliance
Assuring adherence to med. schedule
Promoting weight gainclinical improvementsinclude growth retardation improvements,decrease hepatospleno, encephalopathy, andimmune sys. !x.
Nutritional mgmt to help !""