Respiratory System

Question 1

What is a Tracheoesophageal fistula, how does it present and what conditions are associated with?

Answer 1

It is the incomplete separation of the primitive foregut
Usually presents as polyhydraminos (because improper swallowing) and failure to pass NG. 85% are associated with esophageal atresia.
Genetic Associations: VACTERL, Potters syndrome (neonate exposed to significant oligo 2ndary to kidney disease); Pierre robin, CHARGE, DiGeorge

Question 2

5 Types of Vascular Rings

Answer 2

Abberant Left Subclavian
Double Aortic Arch
Right Aortic Arch
Anomalous inominante artery
Pulmonary artery sling

Question 3

Congenital Diaphgragmatic Hernia

Answer 3

Mostly unilateral and occuring on the left hand side. Associated with Trisomy 13 and 18.
If they are not present on antenatal ultrasound, then usually scaphoid abdomen, medistinal shift.
Biggest risk is associated pulmonary hypoplasia leading to pulmonary htn. If they have to be put on ECMO it is increased M and M.

Question 4

ARDS

Answer 4

Defined as occurring within 7 days of an event and there are new opacities noted on CXR and a PaO2/FiO2 <200.
it is not caused by pulmonary edema or CHF.

Question 5

OI Requirements

Answer 5

OI = (FiO2 *MAP)/PaO2 - how much oxygen and what mean arterial pressure is required to maintain an appropriate PaO2. 
Severe = >16

Question 6

Cystic Fibrosis

Answer 6

Carrier Rate: 1/25. F508 Mutation; It is a mutation in the CFTR carrier - this is an apical epithelial chloride channel.
AR - requires 2 mutations.
Newborn Screen: Immunoreactive Trypsinogen - there is an increased release of trypsinogen from pancreatic ducs secondary to blockage.
False positive: meconium ileus; False Negative: Stress at birth

Sweat Chloride: >60 and >2 kg
False negative: technician error, edema, steroids, decreased sweat production

False Positives; Hypothyroidism; adrenal insufficiency, improper location, g6pd and celiac

Pathophysiology: results in increased Na transport and decreased CL transport.

Question 7

Systems affected by CF

Answer 7

Lung: Allergic bronchopulmonary aspergillosus (can also be seen in severe asthmatics); chronic colonization (pseudomonoas, stenotrophimonas, s. aureus); hypoxemia leads to pulmonary htn; bronchiectasis and nasal polyps
GI: meconium ileus, fat soluble vitamin malabsorption, steatorrhea
Liver: Blocked ducts and fibrosis
Reproductive
Pancrease - enzymes; DM
Bone - osteoporosis secondary to vitamin D defc

Testing: Fecal Elastase, Sweat Chloride, ImmunoTryp (newborn)

Question 8

Asthma - Diagnosis and Clinical Symptoms

Answer 8

Inflammatory disorder of the airways that is characterized by airway hyperresponsiveness to endogenous and exogenous stimuli.
If both parents have asthma there is a 60% chance the child will; one parent - 20%.
All will have onset prior to 7 years of age; most as infants and 2ndary to viral exposure
Diagnosis:
Fev1/FVC <0.7
FEV1 improved by 12% after bronchodilator.

Question 9

Pleural Fluid Analysis - Light’s Criteria

Answer 9

Need Serum and Pleural - Protein and LDH

1. LDH >2/3 normal
2. Protein >0.5 pleural/serum
3. LDH >0.6 pleural/serum

This means exudate: empyema: pneumonia, TB, malignancy or chylo
Transudate: CHF, Cirrhosis, nephrotic, upper airway

Question 10

Pulmonary hemosiderosis

Answer 10

Decreased serum iron levels, hemoptysis, micocytic anemia.

This is a diffuse lung disease with anemia and hemosidin macrophage.

Question 11

5 complications of prolonged OSA

Answer 11

Hypertension, right ventricular hypertrophy, polycythemia, cor pulmonale, pulmonary hypertension

Question 12

5 causes of clubbing (not including hypoxemia)

I CATT

Answer 12

Hepatic: Cirrohsis, alpha antitrypsin
Endocrine: Thyroid
Heme: Thall
Idiopathic

Question 13

Allergic Rhinitis

Answer 13

This is the most common disease of childhood
Clinical presentation: allergic facies (open mouth, tired); allergic shiners, allergic crease, cobblestoning of back of throat, boggy nasal mucosa
Treatment: Environmental Control - mainstay treatment - not always feasible.
Pharm: Antihistamine, nasal corticosteroids and montelukast
Immunotherapy

Question 14

Primary Ciliary Dyskinesia

Answer 14

DHA something mutation. AR
Ciliary immobility, dyskinesa or aplasia.
Diagnosis: Genetics and ciliary motion from biopsy and electron microscopy. Low nasal NO

Presents with recurrent infetions in upper and lower airways. Symptoms are often worse in the mornings. Sinuses often fail to form. (frontal and sphenoid).
Men can be infertile

Question 15

Asthma treatment plan

Answer 15

6-11 years: Low dose Corticosteroid (200 mcg/day)

2. Increase to moderate dosing
3. Add LABA or LTRA (leukotriene receptor antagonist)

> 12 years:
Low dose 250 mcg/day
Add LABA
Add LTRA

Question 16

Asthma ICS

Answer 16

Fluticasone - Flovent
Beclomethasome - Qvar
Ciclesonide - Alvesco 
Pulmicort (Budesonide)
Combined:
Advair (salmeterol and Fluticasone)
Symbicort (formeterol and budesonide)

Question 17

False positive for Sweat Chloride

Answer 17

Hypothyroidism
Location of the site used
Adrenal insufficiency
G6PD 
Celiac

Question 18

False negatives

Answer 18

Not enough sweat produced
Weight
Technician error
Edema (number one cause)