Chronic Disease

Question 1

Cystic fibrosis genetics and epidemiology:

Answer 1

Autosomal recessive; whites; boys = girls

Question 2

What are two findings that suggest CF in both kids and adults?

Answer 2

Kids: Nasal polyps under 12
Adults: Allergies

Question 3

What are three metabolic findings of CF?

Answer 3

• Hyponatremic hypochloremic dehydration
• Metabolic alkalosis
• DM

Question 4

What are the two options for the newborn screen?

Answer 4

IRT

DNA

Question 5

What number is a positive sweat chloride?

Answer 5

60 mEq/L

Question 6

What two things should you supplement a CF patient with for diet?

Answer 6

• Fat soluble vitamins (DEAK)

- Pancreatic enzymes

Question 7

Median age of survival:

Answer 7

40

Question 8

Most common rheumatologist disease:

Answer 8

Juvenile Idiopathic Arthritis

Question 9

What is the race that is most commonly affected by JIA? Age distribution?

Answer 9

Native Americans; BIMODAL (1-3), (8-10)

Question 10

Who is affected more with JIA?

Answer 10

Girls 2-3x more

Question 11

What can be a finding in all types of JIA? What increases the risk for uveitis?

Answer 11

Uveitis - circumlimbal; if ANA positive you are at increased risk for this

Question 12

Symptoms must be present for how long to diagnose JIA?

Answer 12

6 weeks

Question 13

Most common form of JIA?

Answer 13

Oligioarticular (4 or fewer joints involved for 1-6 months)

Question 14

Two types of oligoarticular JIA?

Answer 14

• Persistent (better): Never develop more than 4 joints, 75% remission
• Extended: 5+ joints involved after the first 6 months

Question 15

What type of joints does Polyarticular JIA affect? What are the two types?

Answer 15

Smaller, symmetrical, 5+ joints in the first 6 months of illness

Rh (-): More common and early in childhood
Rh (+): Adolescent females, resembles RA

Question 16

Type of JIA associated with the most mortality or morbidity:

Answer 16

Systemic, fever spikes 1-2x daily for at least two weeks (may accompany or proceed the joint pain)

Question 17

What will occur with the fever spikes in those with systemic JIA?

Answer 17

A discrete salmon colored macule pattern on the trunk and extremities

Question 18

What is Enthesitis Related arthritis (JIA)?

Answer 18

Inflammation where the tendon inserts at the bone - to Dx need at least (2)

Question 19

What are two other findings in those with psoriatic arthritis (JIA)?

Answer 19

• Oncycolysis - nail pitting

- Dactylitis - swelling

Question 20

Peak onset for those with Enthesitis-Related arthritis:

Answer 20

Middle childhood

Question 21

What imaging study can be helpful with JIA?

Answer 21

MRI; but a clinical diagnosis so not needed

Question 22

Initial mild treatment for those with JIA:

Answer 22

NSAIDS

Question 23

For additional JIA management, what else can be used? (3)

Answer 23

• Methotrexate (anti-RA)
• Etanerecept (tumor necrosis)
• Corticosteroids - for the eyes and injections for the joints (not long term)

Question 24

When do detection of DM1 antibodies show up and when do you see destruction and clinical presentation?

Answer 24

Antibodies circulate 5-10 years before clinical presentation

- Clinical signs show up when 80-90% of the beta cells are destroyed

Question 25

Type1 disease onset and epidemiology:

Answer 25

Bimodal: 4-6, 10-14 Boys and girls are equal Common in whites

Question 26

Type2 disease onset and epidemiology:

Answer 26

- Girls | - American Indians --> Pacific Islanders --> AA

Question 27

Four criteria for the diagnosis of diabetes:

Answer 27

1. A1C >6.5% 2. Fasting plasma glucose >126 3. Two hour glucose >200 4. Random >200 + crisis

Question 28

When do you screen for DM?

Answer 28

Every 3 years starting at age 10 or when they hit puberty

Question 29

You are at risk for diabetes with the following four things:

Answer 29

1. FH 2. At risk race 3. Acathosis nigricans, dyslipidemia, HTN, PCOS 4. Maternal gestational diabetes

Question 30

Glycemic control can be more difficult during what three things?

Answer 30

- Exercise - Adolescence - Acute illness

Question 31

Initiate kids on insulin with T2DM when any of the four apply:

Answer 31

- DKA - Glucose levels >250 - A1C >9% - When you can't tell if they have type 1 or 2

Question 32

BMI > ___ = obese | BMI > ___ = Overweight

Answer 32

``` >95% = Obese >85% = Overweight ```

Question 33

What is the main reason kids gain weight?

Answer 33

Reduced energy expenditure

Question 34

Highest risk factor to a child for obesity under the age of 10:

Answer 34

Parental obesity

Question 35

What ages do kids add on fat cells for life?

Answer 35

3-7

Question 36

The majority of childhood obesity is from:

Answer 36

Exogenous causes - energy intake vs. expenditure

Question 37

What are three endocrine issues that can cause obesity?

Answer 37

- Hypothyroidism - Cushing's - GH def.

Question 38

What are three things obesity causes?

Answer 38

- Early menstruation - CAD - OSA

Question 39

When should you start anticipatory guidance for obesity?

Answer 39

6 months

Question 40

Digital screen recs:

Answer 40

<2: None 2-5: 1 hr a day >6: Use discretion

Question 41

Age 2-11 weight loss and >11 weight loss limits:

Answer 41

2-11: No more than 1 pound/week | >11: No more than 2 pounds/week