Hematology Flashcards
RBCs at birth
- high level of erythropoietin
- elevation of # of RBCs
erythropoietin
hormone secreted by the kidneys that increases the rate of RBCs production in response to drop in O2
When breathing starts…
increased level of O2 which then starts to decrease erythropoietin
2-3 mths, levels of RBCs…
continue to drop
-increased risk of developing anemia
Adult levels of RBCs reached…
adolescence
Adolescent boys…
have higher RBCs then girls
Norm Adult male RBC
4.6-5.9 mil
Norm Adult female RBC
4.2-5.4 mil
Norm Children RBC
4.6-4.8 mil
WBCs at birth
count is the highest
-values begin to decline after 12 hours of life and continue for the 1st week of life
1 wk of age, WBC..
begin to stabilize and remain steady until 1 year of age
After that, WBC slowly decreases until the adult value are reached in adolescence
Pediatric Differences in Platelets
- levels are lower in NB than older children and adults
- levels of many clotting factors are also lower in infants
- VIt K is required for synthesis of several clotting factors….prophylactic inj of vit K at birth
Iron Deficiency Anemia
- most prevalent nutritional disorder and most common mineral disturbance in the US
- need iron to synthesize hemoglobin
- iron is lost in stool so need adequate intake
Children 12-26 mths at risk for iron def anemia…
due to cows milk being main staple in this age group
FT infants exhaust iron stores by…
6 months
PT infants exhaust iron stores by…
3 months
Adolescents and Iron def anemia?
rapid growth and poor eating habits
Symptoms of Iron Def Anemia
- pallor
- fatigue
- irritability
- loss of appetite
- decreased physical activity
Prevention of Iron Def Anemia
- family education
- PT infants: use iron supplement at 6-8 wks, continue to age 1
- Formula infants: use iron fortified formula and cereals
- BF infants: after 6 mths, use iron fortified cereals and iron rich foods. some may need iron replacement
- diet education for teenagers, esp females
Iron administration
Iron 4-6 mg/kg for 3 months
- ferrous sulfate better absorbed
- give in 3 divided doses between meals when gastric aids are highest
- also give with juice (vit C, ascorbic acid)
Iron Side Effects
- liquid iron can stain teeth, use straw or dropper towards back of mouth
- vomiting and diarrhea may occur, then give with meals and/or dose reduced then gradually increase
- stools will turn a tarry green color
Sickle Cell Dz
- autosomal-recessive disorder affecting normal RBC development
- 25% chance with both parents carriers
- AA: 1 in 12 blks are carriers, hispanics, west africans
- Replacement of normal hemoglobin with Hgb S
Sickled RBCs
- rigid, inflexible, can occlude small vessels
- increased destruction, life expectancy in 10-20 days
- vascular occlusion=tissue ischemia and organ damage
Sickling causes…
- hemolytic anemia
- organ damage
Hemolytic anemia
- pallor
- fatigue
- jaundice
- irritability
Organ damage
- enlarged spleen
- enlarged liver
- renal insufficiency
- gallstones
- priapism
- abdominal pain
- skin ulcers
Sickle Cell and Spleen..
-splenic sequestration from trapping of blood in spleen
Spleenectomy
- severely compromised immune systems leading to increased rate of infections
- bacterial infections are #1 cause of death in young children
Causes of Sickle Cell Crisis
- infections
- dehydration
- acidosis
- physical exertion
- stress
- exposure to cold
- anything that increases body’s need for oxygen or alters transport of oxygen
Manifestations of Sickle Cell
- depends on the severity of dz
- usually not manifested in first 5-6 mths of life
- anemia and fatigue
- infections and fever
- acute pain with decreased ROM
- jaundice
- hematuria
- splenomegaly
- hepatomegaly
- priapism
Complications of Sickle Cell
- CVA
- Acute Chest syndrome
- hand-foot syndrome (dactylitis)
- blindness
- multiple organ failure
- avascular necrosis
Management of Sickle Cell
-prevent complications, primary prevention
- NB screening
- folic acid
- hydration
- vaccinate
- prophylaxis with PCN or Amoxil until age 5
- annual transcranial doppler for 2-16 yr olds
- assessment for G/D delays
- parent teaching with focus on nutritional and fluid needs plus S/S of infection
- genetic counseling
If doppler results indicate stroke risk…
regular transfusions
Management of Crisis
- BR with BRP-rest
- hydration-oral and IV
- electrolyte replacement-metabolic acidosis
- pain control
- oxygen
- transfusions
- antipyretics
- antibiotics
Parental Education
- seek early intervention for problems
- adequate hydration, specify amount of intake
- avoid hot weather
- watch for signs of reduced intake, decrease UOP, increased thirst
- Prevent infection, immunizations esp flu, pneumo, mening, PCN
- seek early intervention for problems such as fever
- avoid high altitude or non-pressurized air flights
Hemophilia A
“Classic hemophilia”
- deficiency of factor VII
- X-linked recessive disorder
- Amounts for 80% of cases of hemophilia
- occurence 1 in 5000 males
Hemophilia A B
less severe form
- christmas disease
- caused by deficiency of factor IX
- accounts for 15% of cases of hemophilia
Pathophys of Hemophilia
- based on too little antihemophilic factor (AHF)
- AHF is produced in the liver and is needed to form thromboplastin
- Lower amounts of AHF directly relate to the severity of disease.
- bleeding can occur anywhere including skin, muscle, joints, GI, intracranial
Diagnosis of Hemophilia
- hx of bleeding episodes
- can be diagnosed through amniocentesis
- genetic testing of family members to identify carriers
- lab findings: low factor VIII levels and prolonged partial thromboplastin time (PTT)
- the platelet count, prothrombin time, and fibrinogen level are all normal
Clinical Manifestations of Hemophilia
- bleeding following circumcision
- prolonged bleeding at umbilicus
- slow, persistent and prolonged bleeding
- joint tenderness with swelling, warmth, and pain
- epistaxis
- bruising
- hematuria
- pallor
Safety/Precaution of Hemophilia
- teach family early recognition of a bleeding episode
- teach family how to manage bleeds - RICE
- appropriate activites to prevent accidents
- avoid IM injections and heel/finger sticks
- med alert
- provide support