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32 Cards in this Set

  • Front
  • Back
Kawasaki Syndrome
-Most common cause of acquire heart disease in children
-Recognized in 1967
-Acute self limiting vasculitis of unknown etiology
-76% in children < 5 years of age
-Boys > girls
-Asians and blacks > whites (9 & 1.5: 1)
Kawasaki – Clinical Manifestations
**Fever >5 days**
Polymorphous rash
Erythema of oral membranes
Indurative edema of hands and feet
Cervical lymphadenopathy
Non-purulent conjunctivitis
Acute and often toxic presentation
Clinical Manifestations contd
Coronary artery aneursyms and ectasia in 15-25% of untreated cases
May lead to myocardial infarction, sudden death, or ischemic heart disease
High risk
Children < 1 year of age
Males
Kawasaki – Laboratory Anomalies
Elevated acute phase reactants

Elevated ESR

Elevated platelets

Elevated WBC count

Elevated liver transaminases
remember the picture of the kids tiny fingers with skin peeling from around the paronychia...
edema desquamation
EKG in 7 yr old with Kawasaki
Kawasaki Treatment (know this slide!!!!!)
-IV Gamma globulin (IV IgG)
2 gm/ kg over 24 hours
-Aspirin
--20 - 25 mg/kg/dose q6hrs
until afebrile 2-3 days
--3-5 mg/kg/day
6-8 weeks until ESR and platelet count is normal
Kawasaki Buzzwords for Exams
-Febrile child, not responding to antibiotics
-Usually a toddler
-Acute fever of AT LEAST FIVE DAYS
-Must have four of these:
Cervical adenopathy
Dry/fissured lips or swollen tongue
Conjunctivitis
Polymorphous exanthem
Erythema/induration leading to desquamation of hands and feet
Kawasaki Buzzwords for Exams
-Dx cannot be made with < 5 days of fever
-One of few reasons EVER to treat a child with Aspirin
I--nitially high dose (80-100 mg/kg/day)
-Cardiomegaly is NOT a sign of Kawasaki
-Easily confused with:
--Drug reactions (no fever)
--JRA (chronic low grade temp, rash when fever peaks)
--Measles (rash different, measles has exudative conjunctivitis)
Rheumatic Fever/Heart Disease
-Second common acquired heart disease in childhood
-Post-infectious connective tissue response in susceptible host
-Latency period of 3 weeks
-Untreated Group A b- hemolytic streptococcus of the pharynx
-Does NOT follow group A strep infections at other sites
-1/3 of patients have a mild, almost asymptomatic pharyngitis
Rheumatic Fever: High risk groups
High family incidence
Children ages 6-15 years
Low socioeconomic status
Jones Criteria: major
MAJOR
Arthritis - 65 - 85%
Carditis - 60%
Chorea - 20%
Erythema Marginatum - 6%
Subcutaneous nodules - 5%
minor
Arthralgia
Fever of 38 C
Elevated ESR, C-reactive protein
Prolonged PR interval
Diagnosis
***Must have evidence of an antecedent strep infection

History of recent scarlet fever
Positive throat culture
Elevated ASO
Streptozyme test
Carditis (know this slide)
-Occurs 1-2 weeks after strep infection
-Inflammation of
endocardium, valves, myocardium, and/or pericardium
-Prior attack increases risks of recurrence

--Mitral Valve - most common
Insufficiency
Congestive heart failure
Stenosis, occurs years later
--Aortic Valve
Insufficiency less common but more severe
Sydenham’s Chorea
St. Vitis’ Dance

Purposeless, choreiform movements

Aggravated by stress

Emotionally labile
Erythema Marginitum
Characteristic rash
Rarely seen in other disease
Nonpuritic, macular rash
Serpiginous border surrounding normal skin
Seen on trunk and proximal limbs
Not face
Accentuated by warmth
Subcutaneous Nodules
Rare
Seen in chronic rheumatic heart disease
Located on **extensor** surfaces of joints
elbows, knuckles, knees, ankles
Sometimes on scalp and spine
Painless, freely movable
0.5-2 cm in size
Diagnosis
2 major criteria and 1 minor criteria
OR
1 major criteria and 2 minor criteria

Plus

Supporting evidence of an antecedent Group A strep infection
Rheumatic Fever: Treatment (know this slide)
Supportive care

Bed rest

Treat strep infection

Anti-inflammatory agents
Aspirin 100 mg/kg/day divided in 4-6 doses
Prednisone 2 mg/kg/day divided in 4 doses
Reserved for severe cases of carditis
Rheumatic Fever Prophylaxis
To prevent recurrence

Penicillin VK 250 mg bid
Sulfadiazine 0.5 to 1 gm qd
Erythromycin 250 mg bid
LA bicillin 1.2 million units IM q 28 days

Until age 21-25
if no carditis
Not in a high risk group*

For life for rheumatic carditis

*teachers, military, medical profession
SBE Prophylaxis (subacute endocarditis)
In addition to Rheumatic prophylaxis IF there is Rheumatic heart disease

-Clinidamycin 600 mg ( 20 mg/kg)
-Cephalexin 2 gm ( 50 mg/kg)
-plus Gentamicin 1.5 mg/kg IV for GI/ GU procedures
2007 SBE Prophylaxis
-Prosthetic cardiac valve
-Previous infective endocarditis
-Congenital heart disease
--Unrepaired cyanotic CHD
--CHD with prosthetic material or device during the first 6 months after the procedure
--CHD with residual defects at or adjacent to a prosthetic patch or device
-Cardiac transplant recipients with valve disease
Buzzwords for Rheumatic Fever
-Murmur “heard best at the apex” = MR (mitral regurg)
-J♥NES help – pneumonic to remember criteria
arthrITIS = major; arthrALGIA = minor
-Rash is erythema MARGINATUM, but arthritis is MIGRATORY
Erythema migrans = Lyme disease
Atherosclerosis
Cardiovascular disease remains the most common cause of death in the United States

Atherosclerosis is a chronic disease process with origins in childhood

The rate of disease progression is determined by the presence of known cardiovascular risk factors
Atherosclerosis
Multiple studies have identified the risk factors that increase the incidence of coronary artery disease
Framingham Study
Bogalusa Study
PDAY (Pathobiological Determinants of Atherosclerosis in Youth)
Muscatine Study

These risk factors include;
Family history of heart disease
Reduced level of HDL – cholesterol
Elevated serum cholesterol level
Hypertension
Cigarette smoking
Impaired carbohydrate tolerance
Lack of physical activity
picture time
Red Flags
Toddler of a normal weight parent
10% risk of obesity

If one parent is overweight or obese
Risk increases to 40%

An overweight adolescent has a 70% chance of becoming overweight or obese adult
Prevention
Most importantly, a healthy diet & increased physical activity for all children, including low-fat dairy products for all children over two years of age.
Low-fat dairy products for children 12 - 24 months who are overweight.
Screening (a fasting lipid profile) - starting at age 2 years and then every 3-5 years - for all children and adolescents with a family history of high cholesterol / high fat levels or early cardiovascular disease.
Prevention
Screening for all children whose family history is unknown or who are overweight, have high blood pressure, or diabetes.
Weight management should be the primary treatment for overweight kids with high lipid levels.
For patients 8 years and older with an LDL concentration greater than 190 mg/dL (or 160 mg/dL with a family history of early heart disease or two additional risk factors present; or 130 mg/dL if diabetes is present), medications should be considered.
A 12-year-old girl presents to your office for the first time with a swollen, painful, erythematous right knee joint. She tells you that her left knee felt and looked similar yesterday, but now feels normal. She also is easily fatigued and has had fever. On physical examination, she has a temperature of 101.7°F (38.7°C), a heart rate of 125 beats/min, a respiratory rate of 24 breaths/min, and a blood pressure of 120/78 mm Hg. Her lungs are clear. On auscultation, you note a 3/6 holosystolic murmur at the cardiac apex with radiation to the axilla.
a. antibiotic therapy with doxycycline
b. anti-inflammatory therapy with aspirin
c. aspiration of the right knee
d. heat, elevation, and splinting of the right knee joint
e. immunotherapy with azathioprine
Anti-inflammatory with aspirin for rheumatic fever
A 3-year-old girl is brought to your office for re-evaluation of a fever that began 6 days ago. Her mother tells you that her daughter's temperature has been as high as 102.2°F (39°C). Her physical examination was unremarkable when you examined her 3 days ago, but today you note injected sclera; cracked, red lips; a strawberry appearance of her tongue; and a swollen, nontender, cervical node.
a. an exercise stress test should be performed as a baseline study
b. aspirin therapy will be used until the fever subsides
c. cardiac involvement may include abnormalities of the coronary arteries or the myocardium
d. echocardiography should be performed to evaluate for the presence of coronary aneurysms
e. immediate treatment with intravenous immune globulin will eliminate the chance of coronary involvement
aspirin, kawasaki