Aquifer - Cardiovascular and Hematology (Part 2)

Question 1

True or false - there is an association between iron deficiency in infancy and later cognitive defects.

Answer 1

True

Question 2

How does ingestion of cow’s milk in young children increase the risk of iron deficiency anemia?

Answer 2

Excessive ingestion (>24 oz/day)
Low concentration and bioavailability of iron
Risk fo occult intestinal blood loss

Question 3

In the setting of mild anemia, what will many providers do first?

Answer 3

Trial iron rather than doing any further workup. If the Hgb recovers to the normal range, this is sufficient evidence of iron-deficiency anemia.

Question 4

2 classification schemes for anemia?

Answer 4

1. Size (MCV - micro/normo/macrocytic)

2. Mechanism (decreased production, increased destruction, blood loss)

Question 5

Why is the size classification imperfect for anemia?

Answer 5

MCV values in children vary with age and certain conditions do not fit neatly into one category

Question 6

DDx - microcytic anemia

Answer 6

Iron deficiency
Thalassemia
Sideroblastic
Chronic inflammation/disease*
Lead poisoning*
*Can be normocytic

Question 7

DDx - normocytic anemia

Answer 7

Acute blood loss
Immune hemolytic anemia
Hereditary spherocytosis
G6PD deficiency
Sickle cell anemia
Renal disease
Transient erythroblastopenia of childhood (TEC)

Question 8

DDx - macrocytic anemia

Answer 8

Folate deficiency
B12 deficiency
Hypothyroidism
Neoplasms
Bone marrow failure syndromes (aplastic anemia, Diamond-Blackfan anemia, and congenital dyserythropoietic anemia)
Liver disease/failure*
*Can be normocytic

Question 9

How does the reticulocyte count indicate mechanism of anemia?

Answer 9

Decreased production - low reticulocyte count
Increased destruction and blood loss - high reticulocyte count (patient is able to adequately make red cells and is trying to compensate for the anemia)

Question 10

DDx - anemia due to decreased production

Answer 10

Iron, folate, or B12 deficiency
Lead toxicity
Thalassemia
Aplastic anemia
Chronic inflammation
Neoplasms
TEC
DBA
Renal disease
Hypothyroidism
CDEA?
Sideroblastic anemia

Question 11

DDx - anemia due to increased destruction

Answer 11

Immune hemolytic disease
Hereditary spherocytosis
G6PD deficiency
Sickle cell disease
Thalassemia
DIC
Mechanical heart valves
Burns
Paroxysmal nocturanl hemoglobinuria (PNH)
Hypersplenism

Question 12

DDx - anemia due to blood loss

Answer 12

Acute hemorrhage
Dysfunctional uterine bleeding (heavy/prolonged menses)
Pulmonary hemosiderosis (hemorrhage)
Goodpasture's disease
GI blood loss (PUD, other GI conditions)

Question 13

Lab findings of iron deficiency anemia?

Answer 13

Low reticulocyte count
Low iron levels
High iron-binding capacity

Question 14

Risk factors for obesity in children?

Answer 14

1. High birth weight
2. Maternal diabetes
3. Having an obese parent (strongest predictor before age 3)
4. Low SES
5. Certain genetic syndromes
6. Early menarche
7. Shorter extent/duration of breastfeeding

Question 15

Sequelae of obesity in children?

Answer 15

1. (Obstructive) Sleep apnea (cessation of breathing for at least 15 seconds while sleeping)
2. Dyslipidemia
3. Hypertension
4. Slipped capital femoral epiphysis (SCFE) - displacement of the femoral head from the femoral neck through the physeal plate
5. DM2
6. Steatohepatitis

Question 16

Characteristics of steatohepatitis in obese adolescents?

Answer 16

Mild increase in liver transaminases, hyperechoic liver on U/S, evidence of fatty infiltration and fibrosis on biopsy

Question 17

When should an underling endocrinological disorder be suspected in the setting of weight gain in children/adolescents?

Answer 17

Endocrine diseases that cause weight gain usually limit growth and lead to short stature, whereas obesity stimulates stature growth and leads to tall stature for age. It also advances bone age and leads to early puberty.

Only 1% of overweight patients have endocrine problems.

Question 18

Dx DM?

Answer 18

HbA1c 6.5+ % OR
Fasting plasma glucose of 126 mg/dL (no caloric intake for at least 8 hours) OR
Two-hour plasma glucose of 200+ mg/dL during an oral GTT following a load of 75 g glucose OR
Random plasma glucose of 200+ mg/dL in a patient with symptoms

(#1-3 must be repeated to confirm)

Question 19

Compare the presentation of DM1 and DM2 in children.

Answer 19

DM2 - more indolent presentation, weight loss is less common, DKA is rare, accidental diagnosis is common

DM1 - more likely to present in early childhood, weight loss and DKA more common, rare to diagnose accidentally

Question 20

Who should be screened for DM2 (children)?

Answer 20

Children who are overweight (BMI >85th percentile) PLUS 2 of the following:

Family history of DM2 in first or second degree relatives
Race/ethnicity
Signs of/conditions associated with insulin resistance (acanthosis nigricans, PCOS, HTN, dyslipidemia)
Maternal hx of DM or gestational DM during child’s gestation

Question 21

Timing of screening for DM2 in children?

Answer 21

Initiate screening at 10 years of age or onset of puberty (whichever is earlier)
Screen every 3 years
Any test may be used

Question 22

Causes of elevated BP measurements?

Answer 22

1. White coat HTN
2. Positioning (arm at side can elevate)
3. Painful stimuli
4. Cuff that is too small

Question 23

Classification of HTN in children?

Answer 23

Normal: <90th percentile
Prehypertension: 90-95th percentile
Stage 1 HTN: 95th-99th percentile + 5 mmHg
Stage 2 HTN: >99th percentile + 5 mmHg

As with adults, adolescents with BP levels >120/80 but <95th percentile should be considered pre-hypertensive.

Question 24

Management of prehypertension in children?

Answer 24

Therapeutic lifestyle changes
Follow-up in 6 months
No recommended diagnostic workup for a secondary cause unless there is a concern for an underlying cause in H&P or family history

Question 25

Management of primary hypertension in children?

Answer 25

Medications (typically reserved for children with stage 2, secondary, or evidence of target-organ effects) Dietary changes (less sweets, added sugars, fats, red meats, sodium) Weight loss if overweight Physical activity

Question 26

Who should be screened for secondary HTN in children?

Answer 26

Umbilical artery or venous access during the perinatal period UTI (renal scarring) Catecholamine excess (pheochromocytoma or neuroblastoma) Family hx of renal disease Coarctation of the aorta

Question 27

What is vasovagal syncope?

Answer 27

Fainting; caused by self-limited systemic hypotension due to altered neurocardiogenic reflexes leading to bradycardia and/or peripheral vasodilation

Question 28

Prodromal symptoms of vasovagal syncope in chidlren?

Answer 28

Dizziness, lightheadedness, sweating, nausea, weakness, and visual changes; many children experience the prodromal symptoms without syncope

Question 29

What is syncope?

Answer 29

Transient, usually brief LOC and postural tone that results from inadequate cerebral perfusion; typically self-limited with unconsciousness for less than 1 minute, followed by gradual return of cerebral perfusino and awakening

Question 30

How should syncope be evaluated initially?

Answer 30

History (duration of symptoms, time of day, last meal, weight changes, activities leading up to the event, patient position, associated symptoms, drug history) Physical: orthostatic BP measurement, detailed cardiac and neurological exams EKG to rule out important arrhythmic causes of syncope such as WPW syndrome and QT syndrome

Question 31

Red flags for syncope?

Answer 31

Family history of seizures, sudden death, MI in family members <30 years of age All syncope associated with exercise or exertion must be considered dangerous and requires a thorough cardiac evaluation Association with chest pain or palpitations, an abnormal cardiac exam or history of cardiac diseaseFacial cyanosis, aura, frothing at the mouth, tongue biting, slow recovery, or postictal drowsiness and prolonged mental status changes or confusion after the event suggest a seizure Syncope in the supine position, prolonged LOC (>5 minutes), convulsion before LOC, warm/flushed/cyanotic skin color rather than pallor and diaphoresis suggest a seizure

Question 32

Most common cause of sudden cardiac death in pediatric patients?

Answer 32

HCM

Question 33

EKG of a patient with HCM?

Answer 33

Left ventricular hypertrophy and/or ST/T wave changes indicative of repolarization abnormalities Note - ~10% will have normal EKG's

Question 34

If there are worrisome elements for HCM in the history of a pediatric patient with syncope or chest pain, what should be done?

Answer 34

Echo

Question 35

What is precordial catch syndrome?

Answer 35

Most common cause of chest pain in adolescents Thought to be a form of MSK pain of unknown etiology Benign condition characterized by sudden, sporadic onset of sharp pain, usually along the left sternal border, which is often exacerbated with deep inspiration Brief, lasts seconds to a few minutes, resolves spontaneously Pain can often be broken with a forced deep inspiration Often not associated with exercise

Question 36

Presentation of costochondritis?

Answer 36

Benign cause of chest pain, but less common | Pain due to inflammation, lasts for hours or days

Question 37

Presentation of GI causes of chest pain (GERD, gastritis, esophagitis)?

Answer 37

Often retrosternal, burning, non-radiating, associated with meals +medication history (non-prescription meds, OC's, meds that cause gastric irritation) Substance use

Question 38

DDx - Chest Pain | children and adolescents

Answer 38

1. Cardiac cause (HCM, etc.) 2. Precordial catch syndrome 3. Costochondritis 4. GI causes (GERD, gastritis, esophagitis) 5. Asthma or exercise-induced bronchospasm

Question 39

Physical exam findings when assessing chest pain in children?

Answer 39

Vital signs BP w/appropriately sized cuff Palpate brachial and femoral pulses for possible coarctation Auscultation supine and standing Fever (infectious etiology - pericarditis, pneumonia, etc.) Thrill, hyperdynamic precordium, murmurs Body habitus suggesting a genetic disorder associated with cardiac disease (Marfan's) Chest wall inspection for signs of injury; reproducible pain implies MSK origin

Question 40

Symptoms and signs of cardiac chest pain in pediatric patients?

Answer 40

``` Pain triggered by exertion or stress Pressure or crushing sensation 10-15 minutes in length Syncope, palpitations Murmur, thrill, hyperdynamic precordium ```

Question 41

Murmurs that should be further evaluated?

Answer 41

Louder than III/VI Diastolic Increases with standing or Valsalva

Question 42

What is Kawasaki disease?

Answer 42

Acute inflammatory panvasculitis of unknown etiology that impacts any blood vessel, although there is a predilection for small and medium-sized vessels, especially the coronary arteries for unknown reasons It is thought that it results from an AI response to a not-yet-identified infectious trigger

Question 43

Epidemiology of Kawasaki disease (age, timing, genetics)

Answer 43

Disease of childhood, with typical patients 15-80 months; 80% of patients with KD are under the age of 5 years Outbreaks follow seasonal patterns Children of Asian descent have a higher incidence

Question 44

Three distinct phases of Kawasaki disease?

Answer 44

1. Acute phase (onset through 10 days) - fever, clinical findings, elevated APRs 2. Subacute phase (10 days-3 weeks) - fever resolves, clinical findings largely subside often with peeling of hands and feet, elevated APRs 3. Convalescent phase (3-6/8 weeks) - all clinical findings resolved, continued serologic evidence of inflammation

Question 45

Diagnostic criteria for Kawasaki disease?

Answer 45

High fever for at least 5 days + 4 of the following: 1. Change in oral mucosa (i.e., strawberry tongue) 2. Extremity changes (redness/swelling) 3. Unilateral cervical lymphadenopathy (least likely to be present) 4. Rash (non-specific, polymorphic) 5. Conjunctivitis (bilateral, non-purulent) Irritability is common Consider other illnesses with similar features before diagnosing KD

Question 46

Lab evaluation of Kawasaki disease?

Answer 46

1. CBC with differential (elevated WBCs, neutrophilic predominance, normochromic/normocytic anemia is common, thrombocytosis is common, usually starting in week 2) 2. Liver enzymes (transaminases can be elevated) 3. Serum albumin (frequently low) 4. Acute phase reactants (ESR and CRP) - a negative ESR would argue strongly against KD; persistently elevated ESR after the fever supports KD 5. UA - sterile pyuria secondary to sterile urethritis is associated with KD (clean-catch will show white cells, catheterized urine may not)

Question 47

Complications of KD?

Answer 47

1. Aspetic meningitis or other CNS manifestations 2. Coronary artery aneurysm (risk with greatest morbidity, main purpose of treatment is to minimize this risk) - all patients should receive a baseline echo during the acute phase 3. Liver dysfunction 4. Arthritis 5. Hydrops of the gallbladder

Question 48

When is the greatest risk for the development of coronary artery aneurysms during KD?

Answer 48

In the subacute phase (beyond 10 days, almost always within 4 weeks of onset)

Question 49

Rx - Kawasaki Disease

Answer 49

Aspirin and IVIG (standard therapy) High-dose aspirin for its anti-inflammatory properties (shortens the febrile course, no effect on development of aneurysms) Low-dose aspirin following defervescence for its anti-paletlet effects - d/c after 6-8 weeks if no coronary artery changes are seen in follow-up echoes (if abnormalities - continue low-dose aspirin indefinitely) IVIG to decrease the incidence of coronary artery aneurysm (single dose over 10-12 hours)

Question 50

Role of other antipyretics, antibiotics, and steroids in treating KD?

Answer 50

Other antipyretics are not usually effective for fever control Ibuprofen antagonizes the irreversible platelet inhibition induced by aspirin Antibiotics have no effect on clinical course Role of steroids is unclear

Question 51

Follow-up care for KD?

Answer 51

1. Cardiology follow-up visit at 1-2 weeks 2. Aspirin: low-dose for 6-8 weeks after febrile phase, parents should watch for warning signs of GI bleeding, such as stomachache or blood in the stool 3. Flu vaccine - children taking aspirin are at risk for Reye syndrome, a potentially fatal illness that causes multi-organ damage if infected with certain viruses, including influenza and varicella. Parents should avoid exposure to anyone with known flu or chicken pox and children should receive the IM flu vaccine.

Question 52

What does ESR measure?

Answer 52

Indirect measurement of fibrinogen elevation (levels of fibrinogen increase with inflammation) -> higher levels of fibrinogen lead to increased cohesion of erythrocytes and a faster sedimentation rate

Question 53

What does CRP measure?

Answer 53

Direct quantification of an acute phase reactant discovered in blood from patients with pneumococcal pneumonia and named because it reacted with the C-polysaccharide on the cell wall of S. pneumo

Question 54

Compare elevation time course of ESR and CRP.

Answer 54

ESR: rises comparatively slowly in response to an inflammatory stimulus and may not return to normal for weeks after clinical improvement CRP: elevation is relatively quick, beginning at 4-6 hours after initial insult, peaking at 36-50 hours, and returning to normal within 3-7 days after the stimulus is withdrawn

Question 55

Compare the specificity of ESR and CRP.

Answer 55

ESR: not specific for inflammation, also abnormal in pregnancy and anemia; low to moderate reproducibility CRP: more specific than ESR, high reproducibility (Similar in cost)

Question 56

DDx - Fatigue | adolescent female

Answer 56

1. Anemia 2. Bleeding disorder 3. Hypothyroidism 4. Depression 5. Substance abuse

Question 57

Causes of anemia leading to fatigue in an adolescent female?

Answer 57

1. Bleeding disorder | 2. Iron deficiency

Question 58

Presentation suggesting anemia due to a bleeding disorder in an adolescent female with fatigue?

Answer 58

Commonly cause menorrhagia (20% of women with heavy periods have a bleeding disorder) Significant fatigue (rapid loss of Hgb) Family hx of anemia ROS negative unless there is severe systemic illness leading to DIC

Question 59

Presentation suggesting iron deficiency anemia in an adolescent female with fatigue?

Answer 59

Typically not as much fatigue (slow decline allows the body to decompensate)

Question 60

Lab tests to check for a bleeding disorder?

Answer 60

``` CBC with platelets, RBC indices, and a smear, reticulocyte count Pt/PTT Platelet function test Factor VIII activity vWF antigen Ristocetin cofactor (aka vWF activity) ```

Question 61

Presentation of hypothyroidism causing fatigue in adolescent female?

Answer 61

Menstrual abnormalities (menorrhagia, shorter cycles) Constipation, weight gain, decreased appetite Cold skin, slowness, fatigue, prefer hot weather to cold, doing poorly in school, coarse hair [Rash on legs and edema of the feet are signs, but more often seen in adults)

Question 62

Most common hereditary bleeding disorder?

Answer 62

VW disease

Question 63

Inheritance pattern of VW disease?

Answer 63

AD w/variable penetrance (Type 1 and 2) More rare Type 3 is AR

Question 64

Symptoms of VW disease?

Answer 64

``` Ecchymoses Epistaxis Menorrhagia Bleeding post-tonsillectomy and dental extractions Gingival bleeds ```

Question 65

Most common type of VWD?

Answer 65

Type I (70%)

Question 66

Dx VW disease?

Answer 66

Careful clinical history is often the most sensitive indicator Lab dx can be challenging - prolonged platelet function or bleeding time and mild prolongation of PTT point to this diagnosis, but this can be normal To confirm, check VW factor Ag and/or platelet function analysis and factor VIII levels

Question 67

Rx - VW disease?

Answer 67

Intranasal or IV desmopressin Sometimes can give human plasma-derived virally inactivated VWF concentrate

Question 68

Dx prediabetes?

Answer 68

Fasting blood glucose of 100-125 mg/dL OR | Hgb A1C 5.7-6.4%