Hematology
Cardiac
Cardiac Defects
Hemophilia and Sickle Cell Disease
Anemia
100

What is the most common bleeding disorder in children? 

von Willebrand disease 

100

What is endocarditis? 

An infection of the inner lining of the heart and the valves that can enter the bloodstream.

(Antibiotics to prevent in high risk patients prior to dental procedures) 


100

When thinking of Congenital Heart Disease (CHD) or heart defects, there are 4 types.  What are those? 

Defects that increase pulmonary blood flow (VSD, ASD, PDA)

Defects that decrease pulmonary blood flow (Tricuspid atresia, TOF)

Obstructive defects (Pulmonary stenosis, aortic stenosis, coarctation of aorta)

Mixed defects (Transposition of great arteries, Truncus arteriosus, Hypoplastic left heart)

100

What is hemophilia A?

Deficiency of factor VIII (Factor 8) 

80% of the cases 

Referred to as the "classic" type

100

Causes of iron deficiency anemia in children 

Premature birth resulting in decreased iron stores

Excessive intake of cows’ milk in toddlers

  • Milk is not a good source of iron.
  • Milk takes the place of iron-rich solid foods.

Malabsorption disorders

  • Poor dietary intake of iron
  • Increased iron requirements (blood loss)

Adolescents: Menorrhagia


200

What is von Willebrand's disease? 

Inherited lack of the von willebrand factor protein characterized by the inability of the platelets to aggregate

200

What are risk factors for dyslipidemia?

Family history

Genetics 

Obesity 

Medications 

Lack of exercise 

200

What is a Ventricular Septal Defect? (VSD)

A hole in the septum between the right and left ventricle that results in increased pulmonary blood flow

200

What is hemophilia B?

Deficiency in factor IX

Less common that the other form 

Referred to as Christmas Disease

200

Why is iron important? 

Hgb production requires iron

low iron=low hgb

300

How do we treat von Willebrand's disease? 

DDAVP


300

What is Kawasaki's disease? 

Acute systemic vasculitis resolves in less than 8 weeks

Also known as Mucocutaneous lymph node syndrome

The cause is unknown but always in children 

3 Phases: Acute, Subacute, and Convalescent

300

What is an Atrial Septal Defect (ASD)? 


A hole in the septum between the right and left atria that results in increased pulmonary blood flow

300

What is Sickle Cell Disease? 

A group of diseases in which abnormal sickle hemoglobin S (HbS) replaces normal adult hemoglobin (HgA)

Sickle Cell Anemia (hbSS)-person inherited sickle cell genes from BOTH the parents.  This is the "classic type". 

Now tested in all 50 states on newborn screenings 


300

The most common type of anemia worldwide 

Iron deficiency anemia 

400

What are the risk factors for leukemia? 

Male 

Genetics

Family history 

400

What are the signs and symptoms of Kawasaki's disease? 

  • Irritability
  • Red eyes without drainage
  • Bright red, chapped lips
  • Strawberry tongue with white coating or red bumps on the posterior aspect
  • Red oral mucous membranes with inflammation including the pharynx
  • Swelling of hand and feet with red palms and soles
  • Nonblistering rash
  • Bilateral joint pain
  • Enlarged lymph nodes
  • Desquamation of the perineum
  • Cervical lymphadenopathy
  • Cardiac manifestations: Myocarditis, decreased left ventricular function, pericardial effusion, and mitral regurgitation
400

What is a Patent Ductus Arteriosus? 

A condition in which the normal fetal circulation conduit between the pulmonary artery and the aorta fails to close and results in increased pulmonary blood flow

Often see systolic murmurs, wide pulse pressure, and bounding pulses

400

What is a sickle cell crisis? 

Can also be called vaso-occlusive crisis

An acute exacerbation of sickle cell anemia 

Sickled cells get stuck in the veins causing a lot of pain 

triggers: dehydration, stress, infection, cold, high altitude




400

If giving an IM injection iron, what injection method should be used? 

Z track 

500

Interventions for a epistaxis 

  • Have the child sit up with the head tilted slightly forward to prevent aspiration of blood. QS
  • Apply pressure to the lower nose with the thumb and forefinger for at least 10 min.
  • Do not pack cotton or tissue into the nostril or ask the child to blow their nose because this could displace the clot. Encourage the child to breathe through their mouth while pressure is being applied to their nose to control the bleeding.
  • Apply ice across the bridge of the nose if bleeding continues.
500

What is cardiomyopathy? 

abnormalities of the myocardium which interfere with its ability to contract effectively. Can lead to heart failure.

500

What is Tetralogy of Fallot? 

Four defects that result in mixed blood flow: Pulmonary stenosis, ventricular septal defect, overriding aorta, right ventricular hypertrophy

500

What are signs of a Sickle Cell Crisis?

Pain & fever are the most common sign

Acute (related to dehydration and decreased oxygen)

  • Severe pain, usually in bones, joints, and abdomen

  • Swollen joints, hands, and feet

  • Abdominal pain
  • Hematuria
  • Obstructive jaundice
  • Visual disturbances
Chronic (lasting effects)
  • Increased risk of respiratory infections and osteomyelitis
  • Retinal detachment and blindness
  • Systolic murmurs
  • Renal failure and enuresis
  • Liver cirrhosis; hepatomegaly
  • Seizures
  • Skeletal deformities; shoulder or hip avascular necrosis


500

Administration concerns when giving oral iron

Give 1 hr before or 2 hr after milk, tea, or antacid to prevent decreased absorption.

Use a straw 

Can have dark stools (tarry green)

May have upset stomach

600

Name 2 signs of Leukemia

  • Low-grade fever
  • Pallor
  • Increased bruising and petechiae
  • Listlessness
  • Enlarged liver, lymph nodes, and joints
  • Abdominal, leg, and joint pain
  • Headache
  • Vomiting and anorexia
  • Unsteady gait 
  • Pain
  • Enlarged kidneys and testicles
  • Manifestations of increased intracranial pressure
600

What side of the heart has the highest pressure? 

The left side (fighting against the body's blood pressure)

600

What is a Coarctation of the aorta? 


A narrowing of the lumen of the aorta, usually at or near the ductus arteriosus, that results in obstruction of blood flow from the ventricle

600

How do we treat Sickle Cell Crisis? 

Fluids

Pain medications

PRBC

Treat any underlying infections 

oxygen if hypoxemic

600

What are dietary sources of iron? 

Infants: Iron-fortified cereals and formula or exclusive breastfeeding

Older children: Dried beans and lentils; peanut butter; green, leafy vegetables; iron-fortified breads and flour; poultry; and red meat

M
e
n
u