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25 Cards in this Set
- Front
- Back
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Defective development of one or both lungs with resulting decreased lung weight and volume Common anomaly, seen in 10% of neonatal autopsies Most often due to ... in uterus, ... (decreased amniotic fluid), or impaired fetal ... -Congenital diaphragmatic hernia -Renal agenesis -Renal cystic diseases -PPROM (premature prolonged rupture of membranes) |
Pulmonary Hypoplasia
space occupying lesions oligohydramnios respiration |
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look at slide 15
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...:
-Congenital or acquired communication between the trachea and esophagus -Esophagus and trachea both develop from primitive ... -Congenital TEF is common: 1 in 2000-4000 live births -Many children with TEF have other developmental anomalies (e.g. Down syndrome, cardiovascular defects) -Symptoms include copious ..., choking, coughing, ... with feeding -Acquired TEF is typically associated with a ... (lung or esophageal ...) -Surgical repair required |
Tracheoesophageal Fistula (TEF)
foregut salivation cyanosis malignancy cancer |
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...:
A mass of lung tissue without any normal connection to the airway system Theory of formation involves an accessory lung bud that develops from the ventral aspect of the primitive foregut Blood supply from ... or its branches (instead of from the ... arteries) ... sequestrations -External to lung ... sequestrations -Within lung -Associated with recurrent infection or bronchiectasis |
Pulmonary Sequestrations
aorta bronchiole Extralobar Intralobar |
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...:
Clinical syndrome caused by diffuse alveolar capillary damage Severe, acute lung injury involving diffuse ... damage, increased microvascular permeability and non- ... pulmonary edema Acute refractory ... -Does not respond to oxygen therapy Annual incidence 75/100,000 in the US High mortality- 30-40%, ... with age |
Acute Respiratory Distress Syndrome (ARDS)
alveolar cardiogenic hypoxemia increases |
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*Acute Respiratory Distress Syndrome (ARDS)*:
May progress to multisystem organ failure Synonyms include -... lung -... (pathologic term) -Acute ... Injury -Acute ... Injury |
Shock
Diffuse Alveolar Damage Alveolar Lung |
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What are the 4 most common causes of ARDS?
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sepsis
pneumonia gastric aspiration mechanical trauma |
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ARDS causes:
Sepsis* Pneumonia* -Viral, mycoplasma, pneumocystis, ... Gastric aspiration* Mechanical trauma (including ... injuries)* Oxygen toxicity Smoke Irritant gases and chemicals Burns Radiation |
TB
head |
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ARDS Microscopic Findings:
Acute Phase -Interstitial and intra-alveolar ... -Mixed interstitial ... -Loss of Type ... pneumocytes -... membrane formation *... -rich edema fluid mixed with remnants of necrotic epithelial cells Organizing Phase -... -Proliferation of Type ... pneumocytes |
edema
inflammation 1 Hyaline Fibrin Fibrosis 2 |
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...:
Also known as hyaline membrane disease of the newborn (HMD) 60,000 cases and 5000 deaths per year in US Associated with -Prematurity (most cases) -Male gender -Maternal diabetes -Caesarean section delivery Caused by deficiency of ... |
Neonatal Respiratory Distress Syndrome
surfactant |
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HMD pathophysiology:
Inadequate ... production leads to high air-water (blood) surface tensions which favor alveolar ... and gas exchange failure in the lung Collapsed alveoli contain fluid with a ... protein content, ... membranes, and ... bodies derived from the surfactant layer of lung |
surfactant
collapse high hyaline lamellar |
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Tests of fetal lung maturity:
Tests performed on fluid obtained via amniocentesis -Lecithin-sphingomyelin (or "L/S") ratio *If the result is less than 2:1, the fetal lungs may be surfactant ... -Phosphatidol glycerol (PG) *The presence of PG usually indicates fetal lung ... -Surfactant/Albumin (S/A) ratio *The result is given as mg of surfactant per gm of protein *An S/A ratio <35 indicates ... lungs, between 35-55 is ..., and >55 indicates ... surfactant production |
deficient
maturity immature indeterminate mature |
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*Clinical features of HMD*
... distress -tachypnea, grunting, flaring, retractions -Bilateral “...” densities on chest X-ray -Can be difficult to distinguish from pneumonia -Severity peaks at 24-48 hours -Development of ... surfactant and treatment of premature infants(or high risk pregnancy mothers) with ... to stimulate surfactant production have reduced the mortality of RDS -Recovery prolonged by barotrauma or oxidative injury |
Respiratory
ground glass artificial glucocorticoids |
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...:
Seen in premature infants requiring at least 28 days of oxygen therapy Becoming less frequent with gentler ventilation techniques, surfactant therapy, steroid therapy Pathologic findings -bronchiolar and interstitial ... -compensatory emphysema of less damaged acini -inadequate alveolar development causes fewer but ... alveoli |
Bronchopulmonary Dysplasia
fibrosis larger |
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Following a vehicular accident with blood loss leading to prolonged, severe hypotension, a 30-year-old man is intubated and placed on a ventilator. He has progressively decreasing oxygen saturations despite an FI02 of 100%. He remains afebrile. He dies 3 days later. At autopsy, the lungs show diffuse hyaline membranes in alveoli, thickened alveolar walls, alveolar macrophages and a few neutrophils. Which of the following pulmonary diseases most likely complicated his course?
1. Bacterial pneumonia 2. Chronic bronchitis 3. Bronchiectasis 4. Viral pneumonia 5. Diffuse alveolar damage |
5. Diffuse alveolar damage
5) Diffuse alveolar damage (or ARDS as it is known clinically) is the final event following lung injury from a variety of serious illnesses or accidents. |
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A 23-year-old woman is found on prenatal testing to have an elevated hemoglobin A1C level consistent with diabetes. Her pregnancy is uncomplicated until the 29th week of gestation, when she has the onset of premature labor and delivers a male infant 24 hours later. Within an hour after delivery the infant is in severe respiratory distress and requires intubation with mechanical ventilation. Which of the following pharmacologic therapies administered to the mother prior to birth could have helped to prevent this infant's neonatal respiratory distress?
1. Hydrocortisone 2. Oxacillin 3. Ibuprofen 4. Lecithin 5. Surfactant |
1. Hydrocortisone
1) She has diabetes mellitus, which inhibits fetal lung development. At 29 weeks, the baby's lungs do not make sufficient surfactant. Corticosteroids administered to the mother help to speed up type II pneumonocyte production of surfactant in the baby. At birth, exogenous surfactant can be given to the neonate. |
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A newborn male infant develops increasing respiratory distress within an hour following an uncomplicated vaginal delivery at 36 weeks gestation. A plain film radiograph reveals near opacification of both lungs. Despite intubation and positive pressure ventilation, the baby dies within two days. At autopsy, the baby's lungs demonstrate extensive pink hyaline membranes. Which of the following maternal conditions is most likely to increase the risk for this infant's respiratory distress?
1. Diabetes 2. Iron defieciency 3. Lupus 4. Preeclampsia 5. Asthma |
1. Diabetes
1)The hyperinsulinism in the baby as a result of the high glucose impedes development of the type II pneumonocytes. By 36 weeks there should normally be sufficient surfactant to prevent hyaline membrane disease. |
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A 30-year-old woman is in the 28th week of an uncomplicated pregnancy when she experiences the sudden onset of severe abdominal pain, followed by vaginal bleeding, then the onset of labor. A girl infant is delivered on the way to the hospital. Within an hour the baby is in respiratory distress and requires intubation and mechanical ventilation. A day later, a chest radiograph shows opacification of both lungs. The baby's respiratory status does not improve. Which of the following histopathologic findings is most likely to be present in this baby's lungs?
1. Neutrophilic exudates in the alveoli 2. Irregular fibrosis with airspace dilation 3. Alveoli with hyaline membranes 4. Diffuse alveolar hemorrhage 5. Interstitial lymphocytic infiltrates |
3. Alveoli with hyaline membranes
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A male infant is born by caesarean section at 30 weeks gestation. Due to increasing respiratory distress over the next hour he requires intubation and positive pressure ventilation. Two months later, the infant is finally taken off the ventilator, but still does not oxygenate normally. Which of the following diseases has this infant most likely developed?
1. Diffuse alveolar damage 2. Bronchial asthma 3. Bronchiectasis 4. Tracheoesophageal fistula 5. Bronchopulmonary dysplasia |
5. Bronchopulmonary dysplasia
5) BPD is a complication of the treatment for neonatal respiratory distress. The positive pressure ventilation with the higher FIO2's, and the prolonged intubation, all contribute. Generally, these are premature infants who had a respiratory complication following birth, such as hyaline membrane disease. |
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A 60-year-old woman develops multiple organ failure 3 weeks following a pneumonia complicated by septicemia. She has been treated with antibiotic therapy and her sputum and blood cultures now show no growth of organisms. She requires intubation and mechanical ventilation, but it becomes progressively more difficult to maintain her oxygen saturations. A portable chest radiograph shows increasing opacification of all lung fields. Which of the following pathologic processes is most likely now to be present in her lungs?
1. Pulmonary arterial vasculitis 2. Diffuse alveolar damage 3. Extensive neutrophilic alveolar exudates 4. Extensive intra-alveolar hemorrhage 5. Widespread bronchiectasis |
2. Diffuse alveolar damage
2) Diffuse alveolar damage (DAD) is the pathologic term for adult respiratory distress syndrome (ARDS) that is the final common pathway for many acute lung injuries. DAD produces increasing interstitial thickening with mixed inflammation and features of an acute restrictive lung disease |
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A male infant is born at term. During the first month of life the infant has had multiple episodes of respiratory distress from pneumonia with Hemophilus influenzae and Enterococcus. A radiograph reveals a localized area of consolidation near the base of the right lung. Angiography reveals that this segment of lung is supplied by systemic, not pulmonary, arterial blood. Which of the following is the most likely diagnosis?
1. Pulmonary sequestration 2. Chronic abscessing pneumonia 3. Congenital pulmonary airway malformation 4. Bronchogenic cyst 5. Cystic fibrosis |
1. Pulmonary sequestration
1) These have an increased risk for infection and are typically supplied by the aorta or its branches |
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A 20-year-old woman gives birth to a term girl infant following an uncomplicated pregnancy. During the first 6 hours of life, the infant exhibits increased respiratory difficulty following feeding. A plain film radiograph shows bilateral pulmonary infiltrates. No masses are noted. A stomach bubble is seen below the diaphragm. Which of the following congenital anomalies is this infant most likely to have?
1. Extralobar sequestration 2. Congenital pulmonary airway malformation 3. Diaphragmatic hernia 4. Tracheoesophageal fistula 5. Intralobar sequestration |
4. Tracheoesophageal fistula
4) The are various forms of T-E fistula, and a fistulous connection may allow swallowed material to pass into the trachea and lungs. |
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Following allogeneic bone marrow transplantation for acute lymphocytic leukemia, a 19-year-old man has worsening respiratory distress for 2 days. He requires intubation and mechanical ventilation. Sputum cultures and a bronchoalveolar lavage fail to reveal an infectious agent as the cause for his worsening pulmonary function. A lung biopsy is performed and demonstrates hyaline membranes lining alveoli along with alveolar macrophages. These findings are most consistent with which of the following diagnoses?
1. Cystic fibrosis 2. Diffuse alveolar damage 3. Goodpasture syndrome 4. Hyaline membrane disease 5. Idiopathic pulmonary fibrosis |
2. Diffuse alveolar damage
2) This description by itself fits with diffuse alveolar damage (clinically known as adult respiratory distress syndrome). It is the final common pathway of many lung insults |
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A 29-year-old woman gives birth to a male infant at 32 weeks gestation. The infant is intubated but cannot be adequately ventilated and dies at 30 minutes of age. The chest cavity opened at autopsy contains lungs that appear quite small. Which of the following is the most likely underlying cause of death?
1. Tracheoesophageal fistula 2. Bronchopulmonary dysplasia 3. Cystic fibrosis 4. Multicystic renal dysplasia 5. Sudden infant death syndrome |
4. Multicystic renal dysplasia
4) The small lungs suggest the diagnosis of pulmonary hypoplasia as a consequence of oligohyramnios from urinary tract abnormalities that decrease fetal urine output. |
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A 25-year-old woman with a twin pregancy at 35 weeks gestation has developed pregnancy induced hypertension. Her attending obstetrician wishes to perform an amniocentesis to assess fetal lung maturity prior to performing a caesarean section. All of the following tests may in this situation except?
1. Fetal fibronectin 2. Lecithin/Sphingomyelin ratio 3. Phosphatidyl glycerol 4. Surfactant/Albumin ratio |
1. Fetal fibronectin
Fetal fibronectin (fFN) is a protein produced during pregnancy and functions as a biological glue, attaching the fetal sac to the uterine lining. The presence of fFN during weeks 24-34 of a high-risk pregnancy, along with symptoms of labor, suggests that the "glue" may be disintegrating ahead of schedule and alerts doctors to a possibility of preterm delivery. Other newer tests for assessing fetal lung maturity include lamellar body count and foam stability index Fetal fibronectin testing may be useful in women with symptoms of preterm labor to identify those with negative values and a reduced risk of preterm birth, thereby avoiding unnecessary intervention |
