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General principles
- normal ranges vary w/age & race
- newborns=higher Hct (45-65%) & reticulocyte count=2 to 8%
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Hemotocrit
- 42=females
- 48=males
- 45-65=kids
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high reticulocyte means what
more of the factory producing=rbc's
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several days after birth RBC production
decreases & Hgb/Hct levels begin to fall until 2-3mos (Hgb:10 Hct:30)=physiologic anemia of infancy
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newborns have a lrg rbc's w/a mean corpuscular vol >94, when does this fall
6mos
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newborns can have an elevated wbc count at birth of what
9.4 to 30K
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how is the wbc count in reference from infant/young child to teens/adults
relatively higher
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what are mostly seen on a smear at birth & in the older child
neutrophils
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what are mostly seen on semar (80%) btwn 1mos & 6yrs
lymphocytes
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what is it when you put blood on a slide & count cells
smear
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platelet count varies from what to what
150-400K
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Increase #wbc's that is 2 standard deviations above the mean count for a particular age
leukocytosis
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what happens if the wbc count is >50,000
leukemoid reaction which is often seen in sepsis & bacterial
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what is defined as an inc in immature cells i:e greater than or equal 5% of immature to mature neutrophil cells
left shift
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what might you see with a left shift
inc in bands, metamyelocytes & myelocytes
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Left shifts may occur in what
- bacterial inf
- trauma
- burns
- surgery
- acute hemolysis/hemorrhage=all hands on deck so to speak
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Absolute neutrophil count calculated as the wbc count/ul*%neutrophils & bands that is below 2 standard deviations below the normal mean standard for age & race
neutropenia
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hemotological do characterized by an abnormally low # neutrophils
neutropenia
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neutrophil count lower limits
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it is blank whether example above is netropenic
tittering
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When can neutropenia occur=more common
extrinsic factors affecting marrow myeloid cells
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when can neutropenia occur=less common
acquired do myeloid & stem cells
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when can neutropenia occur=rarely
intrinsic defect affecting proliferation & maturation of myeolod & stem cells
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what are some examples of causes of neutropenia
- Infections=viral(HIV) Bacterial (sepsis)
- drug induced-chemo
- PCN
- phenothiazines-given toxic amt
- malignancy-neuroblastoma, rhabdomyosarcoma, ewings sarcoma
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Bone marrow failure
failure to produce an adequated # circulating blood cells that may lead to pancytopenia (dec all types cells) as in aplastic anemia or cytopenia 1 cell line
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bone marrow failure causes
- malignancy
- drugs & toxins
- associated=macrocytosis
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Hereditary clotting Do's
Hemophilia VIII=A or IX=B
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Most common severe bleeding disorders
hemophilia VIII or IX
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Do the factors VIII or IX cross the placenta
no they dont
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When do the symptoms of hemophilia A or B present typically
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hemophilia typically will present as what
intracranial bleed so 30 to 50% affected males bleed w/circumcision which may go undiagnosed
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when could hemophilia show its nasty self
- when they begin to crawl or walk
- sustains the typical minor injuries
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Even in severe cases only blank will have evidence of inc bleeding by 1yr
90%
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Hallmark manifestations of hemophilia
hemarthrosis
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hemarthrosis
blood in ankle joint
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recurrent bleeding in same joint
target joints
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where can life threatening bleeds occur into what structures
- vital such as CNS, upper airway, exsanguitnation
- external=gi, iliopsoas hemorrhage
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patients w/factor VIII or IX usually won't have spontaneous bleeding when
>5U/dl
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patients w/factor VIII or IX may experience prolonged bleeding with what
- dental work
- surgery
- mild-moderation trauma
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how do you dx hemophilia
labs=ptt=affected & maybe 2-3 times normal
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What is the tx for hemophilia
- injury prevention
- hepb vaccine
- pyshosocial counseling
- clotting factor & fluid replacement=severe
- fibrin glue=circumcision=mom carrier must wait
- if no child is carrier=be careful w/circum
- Desmopressin antifibrinolytic=tranexamic acid eposolon aminocarproic acid
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most common hereditary bleeding do chromosome 12
von willebrand disease
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Etiology of von willebrand disease
- varies but some reports suggest 1-2% gen pop affected
- more women than men
- vwf protein: 1=red but not absent; 2=qualatively; 3=abscent
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Symptoms of von willebran disease
- mucocutaneous hemorrhage
- stress
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excessive epistaxis, menorrhagia & post op bleeding
mucocutaneous hemorrhage
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what increases the protein factor & therefore may not have significant bleeding w/childbirth & appendectomy
stress
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how do you dx von willebrand disease
- quanitative assay=VWF antigen, activity
- determination of VWF structure & platelet count
- labs will show inc bleeding & ptt
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how do you tx von willebrand disease
- aim is to inc plasma level of VWF & factor VIII
- DDAVP=desmopressin induces the release of VWF from endothelial cells=milder forms. Otherwise:replacement therapy
- antifibrinolytic therapy=role too
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characterized by ineffective erythropoiesis where there is active erythropoiesis but with premature death of cells, a dec output of RBC from the bone marrow=anemia
megaloblastic anemias
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megaloblastice anemias
- folic acid def
- vit B12 def
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what are some typical findings w/MA
- rbc's larger inc mcv & freq oval
- hypersegmented neutrophils=smear perhaps
- vit def malnutrition is uncommon in us but problem worldwide
- not problem in inner city kids b/c of easy access to food so they get their fortified vitamins
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Folic acid deficiency definiton
folates are found in green veggies, fruits, liver & kidney. body stores for a short time. anemia may occur w/in 2-3mos of folate free diet.
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Etiology of folic acid def
inadequate dietary intake, dec absorption, acquired or cognential do of metabolism
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Findings associated w/folic acid def
- VLBW infants, incidence peaks=4 to 7 mos
- sym=similiar to other anemias w/addition of irritable, poor wt gain, chronic diarrhea. In most sever cases, thrombocytopenia may occur=hemorrhages
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how do you dx folic acid def
labs: consistent w/megaloblastic anemia plus a dec in serum folic acids 3ng/ml. RBC folate levels are more indicative of chronic disease
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normal folic acid levels
5-20 ng/dl
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how do you tx folic acid def
- Folic acid po/IM 0.5 to 1.0mg/day
- improve=72hrs
- transfusion=ill or severe
- long term=MV
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Derived from cobalamin in foods (animals) 2nd production by microrganisms. Human digestion=release & absorption of cobalamin
vb12 def
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Etiology of VB12 def
- inadequated dietary intake or problems w/in dig sys
- vb12=3 to 5yrs in older child & adult; however if a child is born to mother w/def the baby may show signs of anemia=4 to 5mos
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what are the findings w/vb12 def
- weakness
- fatigue
- FTT
- irritability
- palor
- glossitis
- V/D & jaundide
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What are the neuro findings w/vb12 def
- paresthesias
- sens deficits
- hypotonia
- seizures
- development delay
- neuropyshciatric changes
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can neuro changes occur w/out hematologic abnormalities
yes they can
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how do you dx vb12 def
- labs; consistent w/MA
- serum vb12<100pg/ml
- LDH=inc
- bilirubin=inc
- inc methylamalonic acid=urine
- schilling test
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how do you tx folic acid def
parenteral vb12 & oral, nasal formulations=wait to stabilized
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Hemolytic disease of the newborn
- abo incompatibility
- rh isoimmunization
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most common cause of hemolytic dis in NB
ABO incompatibility
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Definition of ABO
most common cause of HDNB, 20-25% preg, only 3rd coomb's pos & only 20% of these develop exc jaundice/hemolytic anemia
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Etiology of ABO incompatibiltiy
- MOM is O & baby is either A or B however if mom=A she may have AB's against B. same principle is true if mom=B
- Mild & present=jaundice
- Also=kernicterus or hydrops fetalis but rare
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how do you tx ABO incompatibility
- phototherapy
- exchange transfusion
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a much less common but can be more severe, severity inc w/each preg, inc abortion-spontaneous or induced
RH isoimmunization
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Etiology of Rh isoimmunization
- 90%=D antigen
- M=Rh neg & baby needs to be Rh pos
- as the baby's antigen enters the maternal circ, maternal IgM Ab's formed=form IgG Ab's
- w/exp/sens IGM, igg ab formation inc, maternal igg ab's can cross placenta=hemolytic disease
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Findings w/Rh isoimmunization
1st preg rarely results in hemolytic disease as sensitization has not recoccured at this time. In severe cases, this may lead to erythoblastotosis fetalis or in less severe cases, hyperbilirubinemai & anemia
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how do you tx Rh isoimmunization=neonatal
- phototherapy
- exchange transfusions
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how do you tx rh isoimmunization=maternal
- rhogam-28 to 32 wks & birth again
- risk of initial sensitization Rh neg mothers has dropped from 10-20% to less than 1% w/rhogam.
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