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Pulmonology > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

CF displays what kind of inheritance pattern?

What PT demographic is most commonly observed w/ CF?

A

Autosomal Recessive (a defunct CFTR gene on chromosome 7)

Caucasians

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2
Q

The CFTR (Cystic Fibrosis Transmembrane Regulatory) gene is responsible for what physiologic function?

If defective CFTR gene is passed to offspring; how does this impact them clinically?

A

CFTR regulates CAMP-chloride transport channels on mucosal surface

Results in increased viscosity of secretions which are difficult to clear

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3
Q

Because of the altered CFTR gene function; what lab could help you clinically Dx CF?

A

SWEAT CHLORIDE! >60mmol/L (due to the altered CAMP-chloride transport; upreg)

If neg. sweat chloride; run DNA analysis to assess for non-classic CF (<2% of cases; but occurs)

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4
Q

What is the clinical presentation of a PT with CF neonates?

A

Meconium Ileus (obstruction of bowel by meconium)

Intestinal atresia (intestinal blockage from stenotic intestine)

Volvulus (Intestine wraps around itself, strangulates itself causing bowel obstruction)

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5
Q

What is the clinical presentation of a PT with CF <1 year of age?

A

Recurrent respiratory infxn or pneumonia
Wheezing and coughing
Steatorrhea (Oily/fatty stools)
FAILURE TO THRIVE

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6
Q

Physical Exam of a CF PT might yield…

A 
Clubbing
Tachypnea, wheezes, crackles
Abdominal Distention
Hepatosplenomegaly
Rectal prolapse
Aquagenic Palms (wrinkled appearance)
Signet ring sign on CXR
Lung Hyperinflation on CXR
PFT's suggesting (OBSTRUCTIVE Dz)
Musculoskeletal:  Scoliosis, kyphosis, lymphadenopathy (parotids or submand.)
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7
Q

Recurrent bronchitis in CF patients may lead to which clinical condition?

A

Bronchiectasis w/ signet ring sign

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8
Q

Airway infections are most commonly observed with which microorganisms most commonly?

A

Staph aureus
Gram neg. bacteria

ULTIMATELY PSEUDOMONAS

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9
Q

T/F

Pancreatic and Sinus involvement does not typically occur in CF PT’s but may present in more severe cases.

A

FALSE

Sinus and pancreatic insufficiency are COMMON!!!

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10
Q

What are some of the possible Pancreatic manifestations of Cystic Fibrosis?

A

Steatorrhea (fatty/oily stool) –> malabsorption of fat can lead to F2T

Abdominal Distention

Deficiencies of Vit ADEK

Pancreatitis

CF related Diabetes

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11
Q

Why do abdominal/GI Px complaints with a CF PT need to be appropriately worked up? What might they be at higher risk of?

A

Small bowel obstruction ~or~ Distal Intestinal Obstructive Syndrome (DIOS)

May require surgery if not caught early enough

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12
Q

T/F

The liver may be cirrhotic in CF patients?

A

TRUE

3rd leading cause of liver transplant for late childhood patients

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13
Q

What musculoskeletal manifestations may present with CF patients?

A

KYPHOSCOLIOSIS

Reduced bone mineral density (order DEXA) if repeated Fx

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14
Q

CF patients require counseling for future family planning because…

A

> 95% of male CF patients are infertile due to dysfunctional sperm motility

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15
Q

What are the common systems impacted by CF?

A 
Sinuses
Lungs
Skin:  Salty/sweaty
Liver
Pancreas
Intestines
Reproductive organs
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16
Q

What is the criteria to make a CF Dx?

A

Clinical S/Sx of 1 impaired organ system, newborn pos. screen, or genetic test pos. of sibiling

Evidence of CFTR dysfunction

  • Elevated SweatChloride x 2
  • 2 CFTR mutations (one each allele)
  • Abnormal nasal potential difference
17
Q

What does a newborn screening include in the US?

A

Two assays
Serum Immunoreactive Trypsinogen (IRT)
DNA analysis for CFTR

18
Q

Sweat chloride levels above what level indicate CFTR testing should be performed?

A

> 60mmol/L

obtained via pilocarpine

19
Q

If you have a CF PT; what specialists should you ensure they have on board as their “case manager’?

A 
Pulmonologist
Gastroenterologist
Surgeon
Endocrinologist
Otolaryngologist 
Cardiologist
Transplant Surgeon
20
Q

Acute exacerbations can be managed with what medications?

A

Inhaled bronchodilators: Albuterol
Chest physiotherapy
Mucolytic: Dornase Alfa (Pulmozyme)
Oral Abx: Fluoroquinolones

21
Q

To manage failure to thrive, fatty stools (steatorrhea), etc. what would you Rx your PT?

A

Pancreatic enzymes (not to exceed 2000 units /kg /meal)

Vitamin Supplements

22
Q

Mucolytics you can Rx a CF PT include???

A

Dornase alfa
Acetylcysteine (Mucomyst)

CFTR Potentiators: (L-I-T)
Lumacaftor/Ivacaftor
Ivacaftor
Tezacaftor/Ivacaftor

23
Q

Antibiotics will be necessary to control CF patients recurrent infections. What are 3 of the more common microorganisms which infect CF patients and what would you use to Tx them?

A

H. influenzae: Chloramphenicol, CEPHALOSPORINS, Piperacillin IV

Pseudomonas: CEPHALOSPORINS, Fluoroquinolone (Cipro), Piperacillin, Aztreonam

S. Aureus: Cephalexin, Cipro, Chloramphenicol, Bactrim

Tobramycin or Gentamicin can also be given for resistant infxns

Pulmonology (17 decks)

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