Disease Pathology Review Flashcards

1
Q

What shunt does Pulmonary Interstitial Emphysema (PIE) cause in neonates?

A

(R->L) shunting. allowing for more hypoxemia.

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2
Q

What is Pulmonary Interstitial Emphysema (PIE)?

A

Leakage of air into the perivascular tissues of the lung from overdistention.

  • can be caused in low birth weight infants on vent support.
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3
Q

Pathophysiology of Pulmonary Interstitial Emphysema (PIE)?

  • what are the snowball affects?
A

When overdistension due to PPV causes ruptures, which allows air to leak into perivascular tissue. Causing:

  • Decreased pulmonary perfusion
  • Increased pulmonary vascular resistance
  • Hypoxemia (due to R->L shunting)
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4
Q

Population affected by Pulmonary Interstitial Emphysema (PIE)?

A

Premature babes

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5
Q

Clinical manifestations of Pulmonary Interstitial Emphysema (PIE)?

A

Sudden idiopathic deterioration

  • bad ABG
  • compliance changes
  • Increasing SOB
  • Increasing vent requirements
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6
Q

Lab findings for Pulmonary Interstitial Emphysema (PIE)?

A

“bubbling” near the hilar region, radiating out.

  • radiolucency’s that are either linear or cyst like
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7
Q

What can happen if a Pulmonary Interstitial Emphysema (PIE) causes air to get trapped in the interstitium?

A

pneumomediastinum, pneumopericardium or pneumothorax.

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8
Q

Management for Pulmonary Interstitial Emphysema (PIE)?

A
  • Try to decrease barotrauma w/min vent support
  • Prone position or lay the worst lung down to improve perfusion
  • HFV (Severe)
  • Selective intubation of good lung (let the bad one recover)
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9
Q

What shunt does Persistent Pulmonary Hypertension (PPHN) cause in neonates?

A

In the presence of high PVR (R->L) shunting.

  • When there is a PDA in the ABSENCE of high PVR or structural cardiac problems, it is described as a L to R shunt.
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10
Q

What is Persistent Pulmonary Hypertension (PPHN)?

A

Failure of high PVR to fall after birth (impaired transition to adult circulation).

  • Pulmonary arterial pressure remains elevated causing a (R->L) shunt through the ductus arteriosus and foramen ovale
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11
Q

Pathophysiology of Persistent Pulmonary Hypertension (PPHN)?

  • what is the snowball affect?
A

Increased PVR, PAP is higher than systemic arterial pressures. More shunting causes a downward spiral of:

  • persistence in severe hypoxemia (refractory) and acidosis
  • pulmonary vasoconstriction and reactivity
  • decreases pulmonary blood flow
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12
Q

Why is the snowball affect of Persistent Pulmonary Hypertension (PPHN) have increasing risk of worsening hypoxemia

A

There is Decreased pulmonary return to the left heart causing a pressure diff between r and l, keeping the foramen ovale open, causing more shunting which leads to the following:

  • leads to pulmonary vasoconstriction and reactivity
  • decreased pulmonary blood flow
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13
Q

Clinical manifestations of Persistent Pulmonary Hypertension (PPHN)?

A

Tachypnea w/mild to moderate respiratory distress and cyanosis.

  • Audible murmur due to aortic regurgitation
  • hypoxemia that is poorly responsive to O2 therapy
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14
Q

Lab findings for Persistent Pulmonary Hypertension (PPHN)?

A
  • Low volume murmur
  • Increased A-a gradient w/marked acidosis and severe hypoxemia
  • CxR’s only if respiratory in origin
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15
Q

Management of Persistent Pulmonary Hypertension (PPHN)?

A

Primary goal is to decrease PVR and increase pulmonary blood flow

  • Ventilate to normal PaCO2 and pH
  • Hyperoxygenation PaO2 >100 to promote pulmonary vasodilation (HFO)

iNO for pulmonary vasodilation

  • Restrict handling and suctioning
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16
Q

What is Ischemia?

A

A condition where the heart muscle does not receive enough oxygen and nutrients.

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17
Q

What is the most common cause of Pneumonia?

A

Strep B

  • Generally bacterial
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18
Q

How is Pneumonia acquired in neonates?

A

Transplacentally from mom to babe in utero, perinatally via contaminated amniotic fluid or passage through colonized vaginal tract, postnatally from exogenous sources.

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19
Q

Pathophysiology of Pnemonia?

A

Exposure leading to:

  • Alveolar infection, filling, pus formation
  • hyaline membrane formation
  • Diffusion defects
  • Inflammation
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20
Q

Snowball affect of a Pneumonia developing in a neonate?

A

Impaired diffusion defects + inflammation + bad gas exchange.

  • Bacterial infection can cause necrosis
  • viral infections stun the cilia and lead to mucus plugging
  • fungal infections can cause cyst formation
  • Reinfections
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21
Q

Clinical Manifestations of Pneumonia?

A
  • Generalized sepsis + low apgars
  • Mom may have Hx of PROM, fever.
  • Generalized listlessness, irritability, poor color and feeding are early signs of infection
  • Tachypnea, cyanosis, grunting, and retractions
  • Thermal instability
  • Apnea
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22
Q

Lab findings for Pneumonia?

A

Blood work may show infection aka increased WBC (Leukopenia)

23
Q

What would a CxR reveal for a pneumonia

A
  • Group B strep: white out, infiltrates, pleural effusions
  • Strep pneumonia: lobar infiltrates, pleural effusions
  • Klebsiella: bilateral consolidation, abscesses
  • Pseudomonas & Serratia: parenchymal consolidation
  • RSV: hyperinflation, patchy consolidation
  • Candida: granular infiltrates, opacity
24
Q

Management for Pneumonia

A

Broad spectrum antibiotic therapy is the mainstay of treatment with fluid therapy and symptomatic control.

  • O2 therapy/mech. vents for severe cases
25
Q

What is Hydrops fetalis?

A

When large amounts of fluid build up in a baby’s tissues and organs, causing extensive swelling (edema).

  • TLDR: Third spaces where there’s not supposed to be fluid and you see fluid.
  • seen in pleural effusions
  • suspected with chest tube so it easier to ventilate
26
Q

What is generalized edema indicative of in neonates?

A

fluid balance [renal] issues, hydrops fetalis, cardiac problems, third spacing

27
Q

What is a Caput Succedaneum?

A

Edema on the scalp secondary to delivery; accompanied by bruising; it crosses the midline of the scalp and is outside the periosteum.

  • looks like a xenomorph head
28
Q

What do Pulmonary Vasodilators treat?

A

Treat increased pulmonary vascular resistance

  • for neonates it’d treat PPHN
  • iNO
29
Q

What is Cystic Fibrosis (CF)?

A

Recessive exocrine gland disorder characterized by excessive viscid (sticky) secretion

30
Q

Pathophysiology of Cystic Fibrosis (CF)?

A

Genetic disease

  • Caused by mutations in the gene that encodes multifunctional protein CF membrane conductance regulator (CFTR)
  • mutation results in thick and tenacious secretions in airway cells, intersinal cells, and exocrine cells
31
Q

What can hyperbilirubin lead to?

A

Elevated bilirubin levels can lead to serious complications, particularly in newborns, where high levels can cause brain damage (kernicterus)

  • Presented as jaudice
32
Q

What is hyperbilirubin lead to?

A

Elevated level of bilirubin in the blood.

  • Bilirubin is a yellow pigment that is produced during the breakdown of red blood cells. It is normally processed by the liver and excreted from the body through the bile.
  • When bilirubin levels become abnormally high, it can result in the yellowing of the skin and eyes, a condition known as jaundice.
33
Q

What is the role of Cystic fibrosis membrane conductane regulator (CFTR)

A
  • CFTR is a protein that plays a crucial role in regulating the movement of salt and water in and out of the cells
  • Affected cells/organs (lungs, pancreas, and digestive tract
34
Q

Clinical manifestations of Cystic Fibrosis (CF)?

A
  • Chronic colonization of Pseudomas Aeruginosa -> often green and smells
  • SOBE and Wheezing
  • Anorexia/weight loss
  • Dry cough progressing to frequent wet coughing spells w/large quantities of secretions
  • Abnormalities in the Respiratory, Digestive and Respiratory tracts
35
Q

Snowball affect of CFTR dysfunction?

A

Decrease in airway surface liquid which impairs mucous clearance.

  • This leads to airway inflammation/infection. Causing chronic productive cough and…Hyperinflation/Bronchiectasis.
  • Endstage CF: pulmonary HTN/Cor Pulmonale
36
Q

What are the most common pathogens associated with cystic fibrosis (CF)?

A

Staph Aureous, Haemophilus Influenza, Pseudomonas Aeruginosa

37
Q

What are Non-respiratory Manifestations of Cystic Fibrosis (CF)?

A
  • Pancreatic Insufficiency: malabsorption of fats and proteins and Failure to thrive in infants
  • Meconium ileus (infants)
  • Reduced fertility (males)
  • Increased risk of Diabetes
38
Q

How is Cystic Fibrosis (CF) diagnosed?

A

Sweat chloride test > 60mmol/L in kids and 29 = CF likely

  • Sodium Chloride concentration in sweat are abnormally high in CF
  • Infant Screening
  • Failure to gain weight
  • Bronchiectasis
  • Recurrent Pancreatitis
  • Often it’s Mom who notices baby’s tear are salty
39
Q

What ranges in a Sweat chloride test indicate cystic fibrosis (CF) in infants 6 months or younger?

A
  • ≤29 mmol/L: Normal (CF very unlikely)
  • 30 to 59 mmol/L: Intermediate (Possible CF)
  • ≥60 mmol/L: Abnormal (CF Diagnosis)
40
Q

What ranges in a Sweat chloride test indicate cystic fibrosis (CF) in infants older than 6 months (kids and adults as well)?

A
  • ≤39 mmol/L: Normal (CF very unlikely)
  • 40 to 59 mmol/L: Intermediate (Possible CF)
  • ≥60 mmol/L: Abnormal (CF Diagnosis)
41
Q

How is Cystic Fibrosis monitored?

A
  • Symptom assessment
  • Physical examinations
  • Regular sputum cultures
  • Spirometry
  • Nutritional Status
42
Q

What are physical therapies that manage Cystic Fibrosis?

A
  • Percussion
  • Postural Drainage
  • BiPAP (during PT)
  • Autogenic drainage
43
Q

How is Cystic fibrosis managed?

A
  • Physical therapy/Bronchial Hygiene
  • Meds
  • Lung transplant
44
Q

What meds are used to manage cystic fibrosis?

A
  • Bronchodilators
  • Inhaled Recombinant Deoxyribonuclease (DNase) (Pulmozyme)– daily in >6 yrs
  • Nebulized 7% hypertonic saline (BID in patients >6 yrs)
  • N-acetylcysteine (Mucomyst)
  • Ibubrofen
  • Azithromycin (patients > 6 yrs)
  • Tobramycin (treat P. Aeruginosa)
45
Q

What kind of disorder is Croup?

A

A subglottic airway obstruction

46
Q

How does Croup affect the larynx?

A

Lower laryngeal, trachea, and occasionally the bronchi are inflamed causing edema/swelling of the mucous a membrane

47
Q

What is the usual cause of Croup?

A

Parainfluenza disease

48
Q

What are clinical manifestations of Croup?

A

barking cough and Steep sign on CxR

49
Q

How is Croup managed?

A
  • Supplemental O2
  • Racemic Epi
  • Corticosteroids
  • Antibiotic therapy
50
Q

What is Epiglottis?

A

Inflammation of the supraglottic region, including the epiglottis, aryepglottic folds, and the false vocal cords.

  • is an obstructive process
51
Q

Clinical manifestations of Epiglottis?

A

Thumb Sign on CxR

  • Drooling
  • elevated WBC
  • Difficulty swallowing
  • no cough
52
Q

What can cause epiglottis?

A

Mostly caused by Haemophilus influenza type b

  • or trauma to the epiglottis (burn)
53
Q

What is management for Epiglottis?

A

Intubate asap

  • supplemental oxygen
  • Racemic epinephrine
  • Corticosteroids
  • Antibiotics