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
What is Hydrops fetalis?
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
What is generalized edema indicative of in neonates?
fluid balance [renal] issues, hydrops fetalis, cardiac problems, third spacing
27
What is a Caput Succedaneum?
**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
What do Pulmonary Vasodilators treat?
Treat increased pulmonary vascular resistance - for neonates it'd treat PPHN - iNO
29
What is Cystic Fibrosis (CF)?
Recessive exocrine gland disorder characterized by **excessive viscid (sticky) secretion**
30
Pathophysiology of Cystic Fibrosis (CF)?
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
What can hyperbilirubin lead to?
Elevated bilirubin levels can lead to serious complications, particularly in newborns, where high levels can cause brain damage **(kernicterus)** - Presented as jaudice
32
What is hyperbilirubin lead to?
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
What is the role of Cystic fibrosis membrane conductane regulator (CFTR)
- 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
Clinical manifestations of Cystic Fibrosis (CF)?
- **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
Snowball affect of CFTR dysfunction?
**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
What are the most common pathogens associated with cystic fibrosis (CF)?
Staph Aureous, Haemophilus Influenza, Pseudomonas Aeruginosa
37
What are Non-respiratory Manifestations of Cystic Fibrosis (CF)?
- Pancreatic Insufficiency: **malabsorption** of fats and proteins and Failure to thrive in infants - Meconium ileus (infants) - Reduced fertility (males) - Increased risk of Diabetes
38
How is Cystic Fibrosis (CF) diagnosed?
**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
What ranges in a Sweat chloride test indicate cystic fibrosis (CF) in infants 6 months or younger?
- ≤29 mmol/L: Normal (CF very unlikely) - 30 to 59 mmol/L: Intermediate (Possible CF) - ≥60 mmol/L: Abnormal (CF Diagnosis)
40
What ranges in a Sweat chloride test indicate cystic fibrosis (CF) in infants older than 6 months (kids and adults as well)?
- ≤39 mmol/L: Normal (CF very unlikely) - 40 to 59 mmol/L: Intermediate (Possible CF) - ≥60 mmol/L: Abnormal (CF Diagnosis)
41
How is Cystic Fibrosis monitored?
- Symptom assessment - Physical examinations - Regular sputum cultures - Spirometry - Nutritional Status
42
What are physical therapies that manage Cystic Fibrosis?
- Percussion - Postural Drainage - BiPAP (during PT) - Autogenic drainage
43
How is Cystic fibrosis managed?
- Physical therapy/Bronchial Hygiene - Meds - Lung transplant
44
What meds are used to manage cystic fibrosis?
- 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
What kind of disorder is Croup?
A subglottic airway obstruction
46
How does Croup affect the larynx?
Lower laryngeal, trachea, and occasionally the bronchi are inflamed causing edema/swelling of the mucous a membrane
47
What is the usual cause of Croup?
Parainfluenza disease
48
What are clinical manifestations of Croup?
barking cough and Steep sign on CxR
49
How is Croup managed?
- Supplemental O2 - Racemic Epi - Corticosteroids - Antibiotic therapy
50
What is Epiglottis?
**Inflammation of the supraglottic region**, **including the epiglottis**, aryepglottic folds, and the false vocal cords. - is an obstructive process
51
Clinical manifestations of Epiglottis?
Thumb Sign on CxR - **Drooling** - elevated WBC - Difficulty swallowing - no cough
52
What can cause epiglottis?
Mostly caused by Haemophilus influenza type b - or trauma to the epiglottis (burn)
53
What is management for Epiglottis?
Intubate asap - supplemental oxygen - Racemic epinephrine - Corticosteroids - Antibiotics