PEDIATRICS FINAL NEW CONTENT

What are the signs and symptoms of physical abuse?

#1 sign is that the history does not match the injury!

Inconsistent story

Distance from parent

Does not protect physical exam

Apathy

Aggressive behavior

Attention Seeking

Cuts, bruises, fractures in various stages of healing

Infected wounds

Spiral fractures, facial trauma/fractures

Infants with fractures

Burns

Bruises (non-accidental sites: back, butt, groin, thighs, backs of knees)

What are the signs and symptoms of cultural practices that can be mistaken as physical abuse?

Cupping

Coining (lines going down diagonally with spine)

Mongolian Spots

Why are infants and toddlers at greater risk for complications from respiratory illnesses?

Smaller trachea diameter

Infant=4mm (1mm edema=50%diameter reduction)

Adult=10mm (1mm edema=20%diameter reduction)

Anatomy of the ribs

Fewer Alveoli @ Birth

Children: 20m/Adults: 300m

Increased Metabolic Demand

Increased RR: Neonates 40-50, Infants 20-40, 5y/o 15-25

Increase Oxygen Consumption

Neonates 6-8mL/kg/min

Adults 3-4mL/kg/min

Locate and identify the anatomical locations for the respiratory and cardiac conditions covered in class.

Condition	Primary Anatomical Location
Epiglottitis	Epiglottis (above larynx)
Croup	Larynx, trachea, bronchi
Pharyngitis	Pharynx
Tonsillitis	Palatine tonsils
Otitis media	Middle ear
Bronchiolitis	Bronchioles
Cystic Fibrosis	Lungs, pancreas, GI tract
Asthma	Bronchi and bronchioles

Condition	Primary Anatomical Location
Murmurs	Heart valves
ASD (Atrial Septal Defect)	Interatrial septum
VSD (Ventricular Septal Defect)	Interventricular septum
PDA (Patent Ductus Arteriosus)	Ductus arteriosus (pulmonary artery → aorta)
Pulmonary Stenosis	Pulmonary valve/outflow tract
Aortic Stenosis	Aortic valve/outflow tract
Kawasaki Disease	Coronary arteries

What are the common s/sx. of laryngotracheobronchitis (CROUP) and how is it best managed?

Hoarseness

Inspiratory Stridor

Croupy/Barky 

LTB

Management: 

Viral (commonly seen in 6 months-4 years) - Cool mist humidifier, wrap in blanket and take outside, steamy bathroom

Bacterial (whooping cough) - make sure children get vaccinated DTaP (2/4/6/15-18/4-6years)

Severe Croup/w Stridor - EMERGENCY treat in ER with epi, corticosteroids, and albuterol

What are common observations when assessing an infant, toddler’s, preschooler’s, school-ager’s, adolescent’s respiratory patterns.

Retractions

Grunting - expiratory sound (usually suggests pulmonary edema)

Nasal Flaring

Wheezing - expiratory sound (#1 cause asthma, then bronchiolitis, bronchitis)

Stridor - high pitched crowing on inspiration (medical emergency, epiglottitis)

Cyanosis - bluish color

Clubbing -

Apnea - cessation of breathing for 20 seconds or more

What are the priority interventions (what order) when you find a child in respiratory distress with increased work of breathing (WOB)?

• 1st - reposition the child > 30 degrees (higher and supine)

• 2nd - check oxygen placement

• 3rd - bulb suction or Mushroom suction the nose

• 4th - then slowly adjust oxygen (infants ⅛ L, ¼ L, ½ L, ¾ L)

• Other interventions

  • Elevate the HOB

  • Supine position

  • Increase the frequency of RT treatments

  • Promote rest - stop over stimulation, organize cares

  • Prevent the spread of infection (nosocomial), contact / droplet precautions if necessary

Which age groups of children and with which conditions/disease processes are at greatest risk for respiratory compromise?

• Infections spread easily from one system to another

• Most commonly viral

• Highest incidence is 3 months - 6 years

  • We seldom see it in children younger than 3 months due to maternal antibodies

• The most serious respiratory infections occur in children < 3 years

• Children are often more ill than they appear

• Croup → common 6 months - 4 years

How is epiglottitis identified? 

• Acute inflammation of the supraglottic structures and the epiglottis

  • The epiglottis is the flap of tissue at the base of the tongue that prevents food from going into the trachea

• True pediatric emergency!!

What assessment findings will be noted for epiglottitis?

• Sudden onset of respiratory distress → over a few hours the status worsens!!

  • Tripod sitting position, stridor, high fever, hoarseness, agitated, and refusing to lay down

• Drooling

What are the interventions for epiglottitis?

• DO NOT VISUALIZE THE UPPER AIRWAY!!!

  • This can cause the epiglottis to spasm which can lead to it worsening and can potentially close off their airway

• Continuous monitoring - stay with the child

• Elevate the HOB

• Continuous O2 sat

• Prepare to intubate

• Diagnosis

  • Clinical signs

    • Sometimes we don’t have time to get an x-ray. We can tell just by the clinical signs that they likely have epiglottitis (tripod positioning, open mouth, drooling, etc…)

  • Lateral neck x-ray

    • Will show a large rounded soft mass at the base of the tongue

What is the pathophysiology for bacterial pharyngitis?

• 90% of sore throats and fevers in children are due to a virus and not bacteria

• Viral pharyngitis:

  • Hand, foot, and mouth disease (Coxsackie virus)

    • Spread by droplet

    • At daycares, we sterilize diaper areas because it can be spread by stool for 8 weeks

    • We need to keep these babies hydrated

  • Pharygoconjunctival fever

• Bacterial pharyngitis:

  • 10% of children with a sore throat and a fever have a Group A Streptococcal Infection

What is the diagnosis for bacterial pharyngitis?

• Rapid antigen tests – very specific, but sensitivity is only 85–90%

  • Positive (+) result = strep

  • Negative (-) result → we need to send a culture to the lab (48 hr return)

What are the assessment findings for bacterial pharyngitis?

• Assessment Findings:

  • Sudden sore throat

  • Fever

  • Headache

  • Anterior cervical nodes

  • Petechiae on the palate

  • Beefy-red uvula

  • Tonsillar exudate 

• If Left Untreated Can Develop into:

  • Acute Rheumatic Fever

    • Carditis – inflammation of the heart

    • Polyarthritis – inflammation of many arteries?

    • Chorea

    • Bright red rash of the trunk and extremities 

  • Post Streptococcal Glomerulonephritis 

Complications of bacterial pharyngitis

• Scarlet Fever

  • The rash is called “macular papular” → that is what you would document if there was a rash that feels like sandpaper.

• PANDAS

  • controversial but possible (essentially strep caused OCD / tics)

  • Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS)

  • Following a strep infection (untreated) is the development of childhood OCD and / or tics 

What is the treatment for bacterial pharyngitis?

• 10 day course of amoxicillin BID

• Carrier State: 10 day course of clindamycin BID

• Surgery

  • Tonsillectomy and Adenoidectomy (surgical removal of the tonsils + adenoid glands)

    • Most common indication is for an obstructed breathing pattern at night

      • Most children with mouth breathing will eventually develop dental malocclusion

      • Maxilla narrows due to the unopposed tongue – called adenoidal facies 

Tonsil Grading

• 0 – surgically removed tonsils

• 1 – tonsils hidden within tonsil pillars

• 2 – tonsils extending to the pillars

• 3 – tonsils are beyond the pillars

  • Starting to intervene at 3 and 4

• 4 – tonsils extended to the midline 

  • 4+ – kissing tonsils (touching)

What is the pathophysiology for cystic fibrosis?

• Autosomal recessive genetic condition

  • If there is a history of CF in an immediate family member, it would be wise to get genetic testing and have the partner get genetic testing as well

• CF gene was identified in 1989 and is located on the long arm of chromosome 7 along with its protein product cystic fibrosis transmembrane regulator (CFTR)

  • Almost 300 alterations that diverge from the original sequence has been reported

  • The delta ΔF508 gene is the most common alteration (~ 70%) and most deadly

• 90% of those homozygous for delta ΔF508 have pancreatic insufficiency, whereas only 36% of CF patients with other mutations have pancreatic insufficiency 

  • The gene seems to lead to both pancreatic deficiency and pulmonary disease

• Since the CFTR is defective, there is an abnormal electrolyte and fluid transport across epithelial cell membranes by mechanisms not well understood 

  • This alteration explains the thickened mucous and secretions of the pulmonary, GI, and reproductive systems

    • Also explains the abnormality in the sweat glands, accounting for the elevated NaCl levels from the blocked transport

What is the diagnosis for cystic fibrosis?

• 1000 infants are diagnosed per year

  • Screen newborns

• Sweat Chloride > 60 mEq NaCl is diagnostic 

  • Gauze pads soaked with pilocarpine is placed on the forearm and small, electric current stimulates sweat glands

• Future Tests:

  • Fecal Fat Test – look for digestive enzymes

  • Followed by Pulmonary Function Tests

    • %FEV1 don’t need to memorize – maria 

      • 75% mild disease

      • 50–74% moderate disease

      • 25–49% moderate to severe disease

      • < 25% very severe disease 

    • 40% is when we put the patient on the lung transplant list. Even if they have a lung transplant, they will still have CF

• Sputum + CF

  • 1. Staphylococcus Aureus 

    • Staph infections are very common in young children 

  • 2. Pseudomonas Aeruginosa 

    • gram negative bacteria – rod appearance

    • This is what turns the mucus green 

  • 3. Burkholderia Cepacia 

    • Grows on potatoes and onions (the bacteria is present when those rot)

      • If we smell rotten potatoes or onions, that is Burkholderia Cepacia

        • If they have this, they will not be put on the transplant list because it could be hiding in their body

What are the assessment findings for cystic fibrosis?

• Respiratory

  • Wheezing

  • Dyspnea

  • Mucousy cough

    • It will be turning green - that’s bacteria

  • Cyanosis

  • Clubbing

    • Lack of oxygen causing inflammation in the small capillaries 

  • Chronic sinus problems

    • Polyps in the sinus cavity - they look like grapes in the nose and they need to be removed frequently.

  • The anterior posterior ratio usually increases with kids with CF because they are retaining air

• GI

  • Meconium ileus at birth (unable to pass first poop)

  • Rectal prolapse (#1 GI sign)

    • Infants are pushing really hard

  • Loose, bulky, frothy, foul smelling stools

    • Frothy and bulky stools happen because they don’t have the pancreatic enzymes and they are passing out fat through their stool

      • Then they can’t gain weight because they are losing all of the stool

  • Increased appetite

    • They will start out with this because they are losing so many calories

  • Increased weight loss

    • As mucous production gets worse, they won’t want to eat (and lose more weight)

  • Vitamins A, D, E, K (fat soluble) are lost

    • A = eyes are affected

    • D = bones are affected

    • E = immunity

    • K = bleeding issues

  • ABDEK or AquADECK (B vitamins added to fat soluble)

• Cardiac

  • Cor Pulmonale (right sided HF due to obstruction of the pulmonary blood flow)

    • Anytime you have a chronic respiratory issue, we will see right sided heart failure

    • Kids with CF should have a chest vest to help shake up the mucous

• Reproductive

  • Males → 98-99% chance of blocking the vas deferens

  • Females → higher percentage of infertility

    • Increased viscosity of cervical mucous (blocks entry of sperm)

What is the treatment for cystic fibrosis?

• Huffing and coughing (huff coughing)

• Autogenic Drainage 

  • Uses different speeds of breathing to move mucus from small airways to medium to large airways

• BPD – bronchial postural drainage 

• CPT – chest percussion treatments (the vest)

  • Rattling 2–3x / day for about 20–30 mins

• Antibiotics 

• Flutter Valve

  • A little “ball” inside the nebulizer that creates a vibration → helps break up the mucus down in the lungs

• PEEP

• Pancrelipase (pancrease) capsules – with all meals and snacks

• ABDECK Vitamins daily

  • ADEK – fat soluble?

  • BC – water soluble?

• Ibuprofen in very high doses

  • Above the 5–10 mg/kg/dose

  • Anti-inflammatory → helps lower the inflammation in the lungs

• Ranitidine (Zantac) 

  • Helps decrease gastric mucosa and resolve those GERD like s/sx

• Pulmonary Function tests to monitor status

  • We are watching this to make sure it is not getting too low

  • < 40% → looking at getting a lung transplant at this point 

• Diet – 130% RDA

• New Drugs

  • Ivacaftor (Kalydeco) by mouth – 1st line (approved in 2013)

    • Treats G551D mutation (4–5% → about 1,200 people in the US)

    • Patient Cost = $236,000 ($450 per tablet)

  • Elexacaftor / tezacaftor / ivacaftor (trikafta) by mouth (approved 2019)

    • 2 years old with at least 1 ΔF508 deletion / mutation

      • Ivacaftor – chloride channel opener

      • Elexaftor / Tezacaftor – are CFTR modulators 

    • Repairs delta ΔF508 deletion / mutations

    • Results in:

      • Increased transport of Cl and Na ions (correcting fluid shifts) and thins mucus secretions

      • ↓ pulmonary exacerbations → 63% lower

        • Seen a drop in hospitalizations for children with CF

      • Sweat Chloride – 41 mEq lower

What is the pathophysiology of asthma?

• Pathophysiology

  • Reversible airway inflammation and increased airway responsiveness to a stimuli

    • Airways are for some reason hyper–responsive to a precipitant or trigger 

  • Condition is manifested by exaggerated bronchoconstriction of the airways 

    • Mast cells in the airways release histamine, which causes smooth muscle contraction + bronchoconstriction. Goblet cells will hypersecrete mucous and there is epithelial damage that leads to ↑ permeability + sensitivity to inhaled allergens and irritants 

  • End Result of Asthma:

    • Airway edema

    • Mucous plugging

    • Significant airway narrowing

    • Leading to rapid airway obstruction 

What are common triggers for Asthma?

• Triggers: be able to identify common triggers

  • Smoke

  • Strong Emotions

  • Furry Pets, dust (can get stuck in carpet → hard flat floors are better), pollen, etc.

  • House dust mites

  • Molds

  • Changes in the weather (ex: I have cold induced asthma)

  • Colds + Viruses → inflammation 

  • Exercises → spasms in the lungs

  • Cockroaches 

    • An insect that turns into dust shortly after death → irritating on the lungs

    • More common in warm, wet environments 

  • Strong Smells → typically adults are more sensitive compared to children 

Atopic March (Asthma)

• Atopic Derm - Allergic Rhinitis - Asthma

• Asthma + Pediatrics

  • Infancy – Atopic Dermatitis / Eczema (1 in 10 children)

    • Reactive Airway Disease → considered the precursor to asthma 

    • Eczema → open skin + bleeding → introduces many different potential asthma triggers 

    • Atopic March 

      • symptoms early in childhood (before the child even has asthma) to assess the risk of the child developing asthma 

        • Stresses the importance of skin barriers like petroleum jelly (vaseline) in the prevention of atopy and future development of asthma

      • Wet-Wraps

        • Dampen the skin first

        • then apply a thick coat of vaseline + wrap with cotton

      • Steroid Creams

        • Good for decreasing inflammation, but thins the skin after long periods of time. Use only for 1 week and then take a break. NOT preferred.

      • Skin Microbiome

        • Research shows: roseomonas mucosa bacterial skin sprays (OTC) have live probiotics that you spray on the skin → improves dermatitis / eczema symptoms 

  • Toddler-Preschool – Allergic Rhinitis

    • The kid who is always witing their nose

    • aka the “Allergic Salute” – a dent in the nose from wiping so often

  • School-Age and Adulthood – Asthma

What are the common cardiac inspections?

• Nutritional status

  • Look at their chest + evaluate if they are taking in enough calories

• Assess Skin (color, mottling, cyanosis in mucous membranes)

  • Mottling in a child: marble appearance of the skin (blotches of red and white in the centers)

  • Mottling is common + expected in the first week of life (prolonged mottling could be a potential concern)

• Excessive perspiration

  • Mild sweating on the brows + lips

• Chest Deformities

  • Pectus Excavatum (Caving in) 

    • Needs to be monitored by a cardiologist to ensure that the heart has enough space to grow and function appropriately 

    • If not the child may need surgery (open heart) to cut open, detach all of the ribs, and then require the whole chest to pop out 

      • This surgery would be done AFTER puberty (ex: a male in his early 20s) and will need good pain management

  • Pectus Carinatum (Coming out)

    • Typically caused by open heart surgery as a child, etc.

    • There is more space for the heart, but usually indicates some sort of cardiac issue

• Retractions

• Clubbing

• Edema 

What are the common cardiac palpations?

• PMI – point of maximum index (i think)

  • < than 7 y/o → @ the 4th ICS (intercostal space)

  • > than 7 y/o → @ the 5th ICS (intercostal space)

• Feel the PMI at the nipple spot

  • If lower, document at the 5th or 6th ICS

  • All we should be feeling is the PMI – your hand should NOT be moving

• Obviously not ideal findings:

  • Lifts →  beating that feels like it’s lifting / moving your hand of the heart

  • Heaves → feels like an up and over motion 

  • Thrills → like a cat purr / vibration

  • Hepatosplenomegaly → can you feel the spleen under the rib cage?

• Check for peripheral pulses 

  • Femoral + brachial 

What are the common cardiac auscultation locations?

• Heart rate and rhythm (listen for 1 full minute, under clothing)

  • Listen APETM

• Components of the cardiac cycle

  • S1 – Mitral and Tricuspid Closure 

    • Coming from the lower portion of the heart

    • Heard better with the diaphragm of the stethoscope

  • S2 – Aortic and Pulmonic Closure 

    • Coming from the upper portion of the heart

    • Heard better with the bell of the stethoscope

    • Systole occurs between the S1 and S2

    • Diasole occurs opposite the pulse

  • S3

    • Normal in children with thin chest walls 

    • Abnormal if > age 30 (indicate hyperdynamic circulation)

    • Sloshing In → sounds like: Slosh (S1) -  - ing (S2) - - - in (S3)

      • Or Kentucky

  • S4

    • Almost always abnormal

    • A stiff wall → sounds like: A (S4) - - Stiff (S1) - - - Wall (S2) 

      • Or tennessee 

How would you describe an innocent murmur, how can this be assessed?  Should you mention to the parent?

• Innocent murmur

  • Consider how and when to mention to the parents

    • Psych-social assessment of parent’s readiness

    • Are you 100% confident it is an innocent murmur?

• Still’s Murmur

  • Most common innocent murmur

    • Soft, low pitched (typically a grade II/VI)

    • Early to midsystolic and musical or vibratory (not just a crisp lub-dub)

    • Heard best at LLSB and the Apex, when the child is supine 

      • PMI (which is at the 4th ICS in children)

      • LLSB = left lower sternal border

      • Listen APETM and then have them sit up and listen again

        • If the murmur becomes louder as the child sits up, there is an increase in the possibility it is no longer “innocent”

        • Now the heart is louder as it’s closer to the rib cage → concerning 

  • Physical Exam Findings for Innocent Murmurs

    • Grade I–II / VI 

    • Systolic in timing (except for venous hum)

    • Musical or vibratory

    • Duration is short (early systolic)

    • LLSB or pulmonic areas

    • Normal Gestation and Delivery

    • Normal S1 and S2

For the congenital cardiac defects covered in class, where are the anatomical defects located? What does this do to blood flow?

ASD Atrial Septal Defect - hole in septal wall between L & R atria/increased pulmonary flow

VSD Ventricular Septal Defect - hole in septal wall between L & R ventricles/increased pulmonary flow

PDA Patent Ductus Arteriosus - left to right shunt until birth (normally closes 24-48 hrs after birth) instead of the blood going to the lungs, can go to aorta for circulation

Left to Right Shunts

• Left to Right Shunts: Acyanotic Congenital Heart Diseases

  • Left-to-right shunts → the pressure pushes blood over to the right side of the heart from the left

    • With acyanotic congenital heart diseases, blood goes from the high pressure of the left side to decreased pressure of the right side, which sends more blood to the lung → leading to overwhelming of the lungs.

      • We will hear wet lungs, tachypnea, sweating while eating, CHF, etc…

Atrial Septal Defect (ASD) - Involves Foramen Ovale

• Left-to-right shunt

• Makes up 10% of those with CHD

• ASD is a murmur meeting:

  • Grade II-IV/VI

  • @ the second left intercostal space (LIS)

  • May radiate to the back

• Will increase pulmonary blood flow + most will need surgery by 3-5 y/o

  • There is not enough pressure within the heart to close off the foramen ovale (after birth), so the blood that already got oxygenated at the lungs is just coming back and repeating the cycle – overwhelms the lungs

    • Pulmonary vascular changes occur after many years if there is no repair and can lead to an enlarged heart

• ALWAYS listen to the top of the heart

  • APETM → the aortic and pulmonic site (top of the heart) is where we are able to identify murmurs and defects like this

Ventricular Septal Defect (VSD)

• Makes up 20-25% of those with CHD (congenital heart diseases)

• The size of the hole determines how much of in increase there is of blood flowing to the lungs

  • Murmur is heard @ LLSB and is grade II-IV/VI

    • The bigger the hole - the quieter the murmur

    • The smaller the hole - the louder the murmur

      • Higher pitched

• You will see pulmonary edema!!

Patients with VSD are at risk for bacterial endocarditis

Patent Ductus Arteriosus (PDA) → Involves Ductus Arteriosus

• Left-to-right shunt → blood is sent back to the lungs again

  • Pulmonary edema!!

  • CHF

  • Left atrial and ventricular hypertrophy!!

  • Sweating during feeds

• Murmur is at the 2nd ICS, under the clavicle

  • Has a machinery type of sound (murmur), with split S2 bounding pulses (sounds like lub de dub).

• Treatment

  • Indocin (indomethacin) - prostaglandin inhibitor

    • Constricts / closes the ductus

    • Education

      • What are also prostaglandin inhibitors? → NSAIDS

      • Educate expecting mothers to NOT take ibuprofen during pregnancy because if it is used, it may close the ductus arteriosus

• Arteriosus of the fetus → unable for blood to shunt & is related to stillbirths

  • Coils in the cath lab will close the PDA

Obstructive Flow - Pulmonary Stenosis 

• Makes up about 8% of those with CHD

• Loud murmur (grade III-IV/VI), @ the LUSB (left upper sternal border)

  • Radiates to the back with a thrill and split S2 sounds (lub-de-dub) 

• Results in right ventricular hypertrophy and decreased pulmonary blood flow

Obstructive Flow - Aortic Stenosis 

• Makes up about 5% of those with CHD

• Murmur is grade II-IV/VI at the 2nd and 3rd ICS

  • Thrill is present + felt at the suprasternal notch

  • Will be able to actually feel the thrill if you place your hand on their chest (upper heart sounds → aortic and pulmonic sites)

• Unless the condition is severe, it may not be picked up until adulthood 

• However, that realization may not get “picked up” early enough because it can also cause → Sudden Cardiac Death!!

  • Lynn had a friend who was in the Marines (clearly a fit guy) who passed out exercising + died → and the mother could only remember a past “sign” being him fainting in the pool one time or something when he was younger  

    • So if we know that they have this condition, educate them to avoid sports → so we can avoid that sudden cardiac death :)

• May also see: 

  • poor pulses due to the stiff aorta → results in difficulty pushing blood out to the body 

  • Becomes fatigue easier than what would be considered normal

  • CXR (chest x-ray) → cardiomegaly 

Coarctation of the Aorta

• Usually occurs near the ductus arteriosus 

  • Left ventricular overload occurs resulting in ↓ BP and weakened pulses in the lower extremities

  • Males 2:1 (more prevalent in men)

• Systolic ejection murmur is present at the left infraclavicular region, that transmits to the back – it is hard to tell where it is actually coming from 

  • Good idea to get an ultrasound → helps visualize 

Right to Left Shunts: Cyanotic Congenital Heart Disease

• With right-to-left shunts, the blood is going right from the right side to the left side without ever going through the lungs. This causes deoxygenated blood to go to the rest of the body and we will see cyanosis in the baby

Tetralogy of Fallot (TOF)

• Ventricular Septal Defect

  • There is no separation between the left and right ventricles (no separation between oxygenated and deoxygenated blood)

  • The defect is high up in the septum → so close to the aorta that the aorta will suck up both deoxygenated and oxygenated blood and push that out to the rest of the body (overriding aorta)

• Pulmonary Stenosis

  • The most important part of TOF!!

  • Blood cannot get to the lungs easily (due to narrowing of the pulmonary artery), so blood takes an easier route.

    • The easier route is to go through the VSD into the left ventricle (the pressure on the right side is so high because of the narrowed pulmonary artery, so it pushes the blood to the left side)

    • Then, it will go from the left ventricle out the aorta to the rest of the body (deoxygenated blood going to the body due to not being able to go through the lungs)

• Right Ventricular Hypertrophy 

  • Blood is not able to exit easily to the lungs due to the pulmonary stenosis → this makes the right ventricle work harder to pump blood out, and this leads to an enlarged heart / hypertrophy

• Overriding Aorta

  • The aorta sits directly over the ASD instead of the left ventricle 

    • Basically the aorta is misplaced and “sucking” blood from both ventricles 

Pulls oxygenated blood from the left side, but it also pulls deoxygenated blood from the right side

“Tet spells” (cyanosis around the fingers and mouth)

• When the baby cries, feeds, or poops.

  • O2 demand increases → right to left shunting increases → MORE deoxygenated blood enters the aorta → the baby turns blue 

• Assessment finding

  • Harsh systolic murmur at LUSB

  • Thrill present

    • Loud S2 → not split because of the decreased pulmonary blood flow

What are the interventions for a child who is having cyanosis, and why does this work?

• When a baby is cyanotic, have them squat!!! 

  • Squatting in a knee-chest position helps re-oxygenate the child because that position compresses the body and stops blood flow to the feet and arms (shunting blood to brain and organs) → increased perfusion!!

    • This is why we tell people to sit down and put their head between their legs when they faint or feel unwell

What is the pathophysiology/etiology for Kawasaki’s Disease?

• First described in 1967 as a mucocutaneous lymph node syndrome

• It is an acute, self-limiting multiple organ system disease of childhood resulting in severe inflammation of blood vessels (coronary arteries)

  • Importance related to the fact 20% of those will develop coronary artery abnormalities 

  • Aneurysms are a big concern because if the coronary arteries get too inflamed, it could cause the vessels to burst which could cause death

• Etiology:

  • Majority of children of Japanese Heritage

    • African American → intermediate risk

    • Caucasians → lowest risk (but actually more at risk in WI)

  • Residence near a large body of water

  • Non-contagious

  • < 5 years old

  • Higher Incidence in the Winter to Spring 

    • Many respiratory illnesses are going around during this time

    • Bellin sees about 2–5 cases / year

    • 5% of those with Kawasaki’s Disease die

What is the diagnosis for Kawasaki’s Disease?

• On clinical grounds only – there are NO blood / lab tests 

  • Assessment findings are our only clue 

  • 5% death rate with this, which is concerning :( 

• Fever for 5 days

• Dry, cracked lips

• Conjunctivitis

• Strawberry tongue, red oral mucosa (papilla are raised)

• Edema of hands + feet

  • Very puffed out → so much it starts to pull the skin away from the nails 

• Pelling of fingertips and toes – “classic sign” -Lynn

• Reddening of the palms and soles of the feet

• Swelling of cervical lymph nodes 

• Patchy rash 

What is the treatment for Kawasaki’s Disease?

• Intravenous Immune Globulin (IVIG) → x 2 doses 

  • Helps prevent an increase in the size of the coronary arteries of the heart 

• High Dose Aspirin (high dose while hospitalized) → then lowered to a therapeutic range (TR) for 2 months of home use 

  • Anti-inflammatory effects in acute phase (bc aspirin is a NSAID)

  • Anti-platelet effects in recovery to prevent blood clots / thrombosis 

• There is a mandatory report to the County Health  Department to track the incidence