Pediatrics Exam Two

classifications of anemias

1. decreased RBC production

-nutritional or bone marrow failure

2. increased RBC loss

-blood loss

3. increased RBC destruction

-sickle cell

management of hematologic disorders

1. treat underlying cause

2. occult blood in stole

3. may need multiple blood draws and heel sticks

- be honest and consider use of emla cream

4. allow rest periods

5. prone to infection -> teach hygiene, crowds and nutition

sickle cell anemia

1. a genetic disorder that causes RBCs to sickle to depranocytes (elongated) under stress

2. they vaso-occulde or get stuck together blocking blood flow

3. they then go through hemolysis or break apart easily (call this RBC destruction) leading to anemia

- common in african americans

- 1 in 4 chance of getting if both parents have

4. will have chronic anemia (Hgb 6-9)

causes of stress that lead to RBCs sickling...

1. dehydration

2. hypoxia

3. acidosis

diagnosis of sickle cell anemia

newborn screening

vasocclusive crisis

painful episode characterized by ischemia causing mild to severe pain that lasts minutes to days

-hard to control pain that is recurrent

-no ice= vasoconstricts more

Sequestration Crisis

large amounts of blood pool in the liver and spleen causing decreased blood volume and hypovolemic shock

signs of hypovolemic shock

1. hypotension

2. tachycardia

3. weak pulses

4. cool extremities

5. poor cap refill

-all are signs of poor perfusion

Acute Chest Syndrome (ACS)

1. check back and abd pain

2. fever

3. cough

4. dyspnea

5. tachypnea

6. retractions

7 declining O2 stats

-from blocked blood flow and inflammation in the lungs

CVA (stroke)

long term complication of unrelieved headache, vomiting, twitching, seizures, inability to move arm or leg, stagger walking, slurred speech and weakness

Factors that precipitate a crisis include...

1. dehydration

2. high altitude

3. cold

4. illness

5. physical strain on the body

Management of sickle cell anemia

1. no O2 unless hypoxic

2. regular blood transfusions for severe anemia (can cause iron overload depositing into kidneys)

3. exchange transfusions-> prevention of CVA

4. splenectomy-> decrease incidence of recurrent sequestrian crises; risk of infection

5. pneumococcal vaccines to reduce infection risk

6. adequate hydration to prevent sickling

7. PCA for pain management of vaso-occlusive crisis

Iron deficiency anemia

low iron-> low hemoglobin -> low oxygen carying capacity of RBCs-> tissue hypoxia

Pts at risk of iron deficiency anemia

1. premature -> supplemented at 2 mos

2. exclusively breastfed -> supplement at 4 months

3. toddlers-> switch to cows milk at 12 months; if do not have enough in diet become deficent

4. teens -> fad diets, poor eating habits, strenuous activities

5. girls-> lose blood during menstural cycles

Diagnosis of iron deficiency anemia

1. decreased hgb/hct

2. decreased ferritin

3. increased TIBC

manifestations of iron deficiency anemia

1. fatigue and activity intolerance

2. pallor and cool skin

3. brittle, spoon shaped nails

4. dizziness

5. headache

6. tachycardia

7. poor appetite

Iron supplements

1. given orally in 2 divided doses between meals

2. best absorbed on an empty stomach in an acidic enviornment

3. can take with fruit juice

4. do not give with milk products

5. liquid iron-> straw or syringe to back of mouth and brush teeth after.

6. check hgb at 1 month, treat with supplements for 3 months

IM and IV iron

IM= Ztrack, do not massage, can stain skin

IV= will give test dose to watch for allergic reactions

Iron stools

dark, greenish black stools

- if not present could indicate noncompliance

- hemocult monitored but not every time get a dark stool

If no improvement in Hgb check for...

bleeding or noncompliance

diet for iron deficiency anemia

1. iron fortified formula or cereal

2. meats (beef, chicken, fish)

3. wholegrain breads

4. vegetables

5. eggs

6. chickpeas

7. nuts

allergic reaction from blood transfusion

1. hives

2. itching

3. difficulty breathing

4. airway closure

hemolytic reaction from blood transfusion

1. blood in urine

2. flank pain

Thalassemia

Inherited Hgb defect that leads to destruction of fragile RBCs

-higher rates in mediterranean descent

early manifestations of thalessemia

1. pallor

2. poor feeding

3. enlarged spleen and liver

chronic signs of thalessemia

1. headache

2. activity intolerance

3. anorexia

4. chest pain

5. bone pain

6. delayed growth

7. enlarged head with flat nasal bridge if untreated

8. small stature

9. delayed sexual maturation

10. bronzed freckled complexion

Treatment of thalessemia

1. transfusions every 3-5 wks to keep hgb > 9.5

2. chelation therapy to prevent iron overload from frequent blood transfusions by removing metal from the body

3. splenectomy for fewer transfusions

-prophylatic antibiotics and vaccines

4. all families should be tested for the trait

considerations for chelation therapy

-side effects= GI upset, headaches, joint pain, renal dysfunction

-promote hydration while on therapy

Hemophilia/Von Willebrand Disease

Inherited deficiency of specific clotting factors causing prolonged bleeding

Manifestations of Hemophilia/Von Willebrand Disease

1. hemorrhage from trauma

2. excessive bruising

3. hematomas

4. hemarthroses -> internal bleeding into joints

-pain, warmth, swelling, decreased ROM

-may cause bone deformities from reccurent episodes

5. hematuria

complications and emergencies from hemophilia

1. bleeding in neck/mouth/chest-> concern for airway obstruction or aspiration

2. intracranial bleed-> headache, slurred speech, LOC changes

3. spinal hematomas-> can cause paralysis

4. GI bleed-> black tarry stools, decreased H&H, abd pain

Main treatment for hemophilia

replacement therapy of missing clotting factor

activity and joint care in hemophilia

1. exercise and physical therapy strengthens joints and prevents bleeding

2. do not do during an active bleed

3. do RICE for hemarthosis (joint bleeding)

what should be avoided in the care of hemophilia?

1. im injections if possible

2. heel sticks

3. aspirin and NSAIDS

4. invasive procedures

Safety and hygiene precautions for hemophilia

1. soft toothbrush

2. water flosser

3. electric razor

4. medical alert bracelet

5. noncontact sports

6. teach older children self-infusion

how to manage an active bleed with hemophilia pts

1. apply direct pressure

2. immobilize and elevate the injured area

3. after bleeding stops, start active rom

HIV

retrovirus attacks CD4 and T-lymphocytes, suppressing the immune system, increasing risk for infections and malignancy

-chronic by manageable with therapy

transmission of HIV

1. perinatal

2. sexual

3. IV drug use

4. blood or bodily fluid exposure

Manifestations of HIV

1. liver and spleen enlargement

2. parotitis (parotid gland swelling)

3. swollen lymph nodes

4. oral candidiasis and trouble eating

5. chronic diarrhea and n/v

6. failure to thrive

7. developmental delays

8. muscle pain

9. rashes

Diagnosis of HIV

- 18 + monthes = ELISA + western blot blood tests

- <18 months = PCR testing

- rapid test results in 30 mins; get confirmed with western blot or another rapid +

AIDS diagnosis

Below a certain CD4 level

associated with certain diseases: pneumocystis carinii pneumonia, lymphoid interstitial pneumonitis,recurrent bacterial infections, wasting syndrome, candida esophagitis, HIV encephalopathy, Cytomegalovirus, Mycobacterium avium-intracellular complex infection, pulmonary candidiasis, herpes simplex disease, cryptosporidiosis

antiretroviral therapy (ART)

drug combo to suppress virus at all stage of HIV cycle

-suppresses viral replication and prevent and prevents immune system deterioration

- CD4 count and viral load done routinely to monitor disease progression and response to therapy

- hard on GI system so check compliance

Management of HIV

1. pneumococal and flu vaccines to prevent infection

2. MMR/varicella only if not currently immunocompromised

3. can attend school or daycare; parents do not have to report

4. should follow standard precautions

5. educate safe sex, drug needles

6. nurses should always wear gloves

Nutrition for HIV

-High calorie, high protein

-may have nutritional issues due to GI symps and oral thrush

Risk factors of congenital heart defects

1. exact cause unknown

2. maternal diabetes

3. maternal PKU

4. alcohol consumption

5. family history of defects

-will get fetal echos

6. chromosomal anomalies such as down syndrome

Acyanotic heart defects

-HF symptoms and pink skin

1. defects with increased pulmonary blood flow

2. obstructive defects

defects with increased pulmonary blood flow

1. Atrial septal defect (ASD)

2. Ventricular septal defect (VSD)

3. Patent ductus arteriosus (PDA)

Obstructive defects

1. Coarctation of the aorta

2. Aortic stenosis

3. Pulmonic stenosis

Cyanotic heart defects

- HF symps and signs of poor perfusion; blue skin

1. defects with decreased pulmonary blood flow

2. mixed defects

defects with decreased pulmonary blood flow

Tetralogy of Fallot

mixed defects

1. Transposition of the great vessels

2. hypoplastic left heart syndrome

Artial Septal Defect

Abnormal opening between the atria, allowing blood to flow higher pressure left atrium and lower pressure right atrium

-causes increased flow of oxygenated blood to right side of heart

-3 types with diff locations of whole

Manifestations of atrial septal defects

1. often asymptomatic with spontaneous close in young pts with small defects

2. larger hole= more symps

3. can lead to heart failure if undiagnosed

4. systolic murmur with fixed second sound

5. risk of developing atrial dysrhythmias, pulmonary vascular obstructive disease, and emboli later in life

Ventricular septal defects

abnormal opening between the ventricles

- small pinhole to absense of septum

-blood flows through defect to pulmonary artery and then pumped into lungs from increased pressure

-will spontaneous close in first year of life in small defects

manifestations of ventricular septal defects

1. Heart failure is common

2. risk of endocarditis

3. loud holosytolic murmur heard at the left sternal border

Patent Ductus arteriosus (PDA)

failure of the fetal ductus arteriosus (artery connecting the aorta to the pulmonary artery) to close within the first weeks of life

-causes right to left shunt

Patent ductus arteriosus (PDA) manifestations

1. amount of shunting determines degree of symps

2. machinery like murmur

3. widening pulse pressure

4. bounding pulses

5. may be asymptomatic

6. may show signs of HF

coarctation of the aorta

narrowing near the ductus arteriosus which results in increased pressure to the upper extremities and decreased pressure to the lower extremities

manifestations of coarctation of the aorta

1. high blood pressure and bounding pulses of the upper extremites

2. weak or absent femoral pulses with lower BP and cool extremities

Infants vs older children manifestations for coarctation of the aorta

1. infant-> HF, severe acidosis and hypotension

-mechanical ventilation and inotropic support may be needed before sugery

2. older children-> dizziness, headache, fainting, epistaxis, hypertension

-risk for ruptured aorta, aortic aneurysm (watch for rupture), and stroke

Postop care for coarctation of the aorta

1. monitor for hypertension

2. may need IV treatment followed by PO of ace inhibitors or beta blockers

aortic stenosis

narrowing of the aortic valve

-decreased cardiac output

-left ventricular hypertrophy

-pulmonary vascular congestion (pulmonary edema)

aortic stenosis manifestations

1. faint pulses

2. hypotension

3. tachycardia

4. poor feeding

5. exercise intolerance

6. chest pain

7. dizziness when standing

8. systolic ejection murmur

9. risk of endocarditis

pulmonic stenosis

narrowing of pulmonic valve

-causes ventricular hypertrophy and decreased pulmonary blood flow

pulmonic stenosis manifestations

1. may be asymptomatic

2. mild cyanosis

3. HF

4. loud systolic ejection murmur

5. cardiomegaly on chest Xray

6. risk of endocarditis

Tetralogy of Fallot

congenital malformation involving four distinct heart defects

- VSD, PS, overriding aorta, and right ventricular hypertrophy

manifestations of tetralogy of fallot

1. acutely cyanotic at birth or cyanosis that progresses in first year of life

2. loud systolic ejection murmur

3. tet spells

4. anoxic spells

5. risk for emboli, seizures, and sudden death

Transposition of the great arteries

Aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle

-septal defects and PDA may present

manifestations of transposition of the great vessels

1. cyanotic at birth

2. depressed function

3. HF symps

4. cardiomegaly

-vary depanding on type and size

hypoplastic left heart syndrome

underdevelopment of the left side of the heart

manifestations of hypo plastic left heart syndrome

1. mild cyanosis

2. signs of HF until PDA closes which leads to increased cyanosis and decreased cardiac output

3. usually fatal

Heart failure

inabillity of the heart to pump sufficient blood to meet body's needs

general symptoms of HF

early= tachycardia, tachypnea, fatigue, irritability

late= hypotension, weak pulses, pale/cool extremities, and decreased urinary output

Right sided HF manifestations

systemic

1. edema

2. enlarged organs *hepatomegaly

3. weight gain

4. JVD (older children)

5. ascites

6. anorexia or GI distress

Left sided heart failure

pulmonary symptoms

1. respiratory distress

2. dyspnea

3. retrations

4. nasal flaring

5. wheezing

6. cyanosis

7. activity intolerance

diagnosis of Heart failure

1. chest xray (cardiomegaly)

2. ECG

3. echocardiogram

Meds given for HF

1. digoxin

2. ace inhibitors

3. beta blockers

-improve cardiac fcn

4. diuretics

- remove fluid and sodium

Management of HF

1. fluid restriction-> space fluids through shift; small cups that are full

2. sodium restriction-> if increased edema; no added salts, soups, salad dressings

3. semi fowlers and energy conservation

4. cool humidified oxygen

5. feedings q 3 hrs, half hour for feeding, increased caloric needs

6. stroke cheek to promote sucking

7. premie nipple to decrease energy

8. if resp rate > 80 hold feedings and use gavage feedings

9. daily weights for retained fluid and abd circumferences for ascites

10. may give potassium-> has to be in pump or mixed if liquid

11. 1 mL/kg/hr urine output

hypoxemia vs hypoxia

hypoxemia is low oxygen in the arterial blood and hypoxia is low oxygen in the tissues resulting in low O2 stat

manifestations of hypoxemia

1. polycythemia -> increased RBCs making blood thicker

2. clubbing of nails

3. tet spells

-rarely seen before 2 mos

- occur in morning after feeding, crying, or stressfull procedures

4. cyanosis with low O2 stats

If a patient is having a tet spell...

1. place in knee-chest position or squat

2. remain calm

3. administer 100% O2 via blowby

4. morphine

5. iv fluids

managing hypoxemia

1. prostaglandin E for vasodilation and smooth muscle relaxation

2. monitor for anemia

3. prevent dehydration by giving Iv fluids as this can increase already viscous blood

4. treat pulm infections with chest PT, antibiotics etc

Postop care for congenital heart defect pts

1. VS for 1 full minute

2. lung auscultation

3. hypothermia expected immediately after

4. A-line may be in place to measure direct arterial blood pressure

-lots of drainage can indicate hemorrhage

5. may have chest tube in place or require suctioning

6. fluid restriction

7. watch HR and RR with activity

Rheumatic Fever

autoimmune response to untreated strep, leading to inflammation of heart joints and brain

diagnosis of rheumatic fever

-Jones' criteria

-- 2 or more major symptoms

OR

--1 major & 2 minor with evidence of strep

- will have elevated ESR and CRP but just indicate inflammation is present

--if improving will come down

Major symptoms of rheumatic fever

1. carditis -> tachycardia, cardiomegaly, muffled heart sounds, pericardial friction rub, new or chaned murmur

2. polyarthritis -> red, hot painful swollen joints

3. chorea -> sudden, irregular involuntray p disturbances

4. erythema marginatum rash -> circular, nonpruritic rash

5. subq nodules on bony prominences

minor symptoms of rheumatic fever

1. fever

2. arthralgia (joint pain)

3. elevated ESR or CRP

4. prolonged PR interval on ECG

evidence of strep

1. positive throat culture

2. rapid strep test

3. elevated ASO strep antibody titer lab test

prevention of rheumatic fever

1. strep tests for culture and treatment if positive

2. treat with penicillin and take all antibiotics until gone

3. long term prophylactic antibiotics to prevent future infections for 5 years or until 21; 10 years to 21 if carditis; 40 yrs or indefinite if carditis and residual heart defect

treatment of carditis from rheumatic fever

1. corticosteroids

2. activity restriction until fever subsides, then can resume full activity

3. adequate rest

treatment for polyarthritis from rheumatic fever

salicylates (aspirin)

infective endocarditis

bacterial infection of the endocardium especially involves the valve tissues

manifestations of endocarditis

1. unexplained fever, anorexia and weight loss

2. splinter hemorrhages-> black lines in fingernails

3. osler nodes-> painful red intradermal nodes on fingers

4. HF, dysrhythmias, or change in murmur

5. petechiae of mucosa, conjunctiva, or extemities

6. janeway lesions-> painless hemorrhagic spots or palms/soles

diagnosis of infective endocarditis

1. blood cultures-> show inf in blood stream

2. echocardiogram -> shows infection in heart

3. ESR/CRP elevated -> go down when symptoms resolve

Treatment of endocarditis

1. high dose IV antibiotics for 8 wks after cultures

2. frequent echos

3. prophylactic antibiotics before procedures

4. valve replacement if unresponsive to treatment

Kawasaki Disease

acute systemic vascular inflammation

acute phase of kawaski disease

1. fever 102-106 unexplained, >5 days, unresponsive to antipyretics

2. irritability

3. increased platelets, increased WBCS, increased LFT and increased ESR/CRP

4. edema, erythema of palms and soles

subacute phase of kawaski disease

1. starts resolution of fever

2. ends with total symp resolution

3. desquamation of palms and soles

-risk of coronary aneurysm, thrombosis, and MI

convalescent phase of kawasaki disease

symptoms resolved but labs do not normalize

-for 6 to 8 wks

diagnosis criteria for kawaski disease

febrile for >5 days with 4 or 5 of these criteria

1. changes in extremities (erythema or desquamation)

2. bilateral conjunctival inflammation

3. erythematous lips or strawberry tongue

4. rash

5. unilateral cervical lymphadenopathy