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FoundSci III > Childhood Diseases III > Flashcards

Childhood Diseases III Flashcards

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1
Q

What are the causes of neonatal respiratory distress? x5

A
  1. Neonatal Respiratory Distress Syndrome
  2. Maternal excess sedation
  3. Fetal head injury during delivery
  4. Blood or amniotic fluid aspiration
  5. Intrauterine hypoxia brought about by umbilical cord coiling about the neck
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2
Q

________ is caused by lack of surfactant due to prematurity. 60% of infants born less than 28 weeks will have this

A

Neonatal Respiratory Distress Syndrome

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3
Q

Neonatal Respiratory Distress Syndrome is also called _______

A

Hyaline Membrane Disease

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4
Q

What is the leading cause of mortality and morbidity in PREMATURE neonates/infants?

A

Hyaline Membrane Disease

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5
Q

Hyaline membrane disease is strongly associated with ________ x3

A

Maternal diabetes
Delivery by C section
Male gender

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6
Q

What is the fundamental defect in neonatal RDS?

A

Deficiency of pulmonary surfactant

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7
Q

Immaturity of the lungs is the most important substrate on which ________ develops

A

Neonatal RDS or Hyaline Membrane Disease

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8
Q

What occurs with decreased alveolar surfactant? x2

A

Atelectasis which causes:
Uneven perfusion
Hypoventilation

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9
Q

Atelactasis that occurs with Neonatal RDS then causes what downstream effects?

A

Hypoxemia + CO2 retention –> acidosis/pulm vascoconstriction/pulm hypotension –> endothelial and epithelial damage

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10
Q

If under a microscope, you see alternating atelactic alveoli and alveolar ducts that are dilated and lined by eosinophilic fibrin-rich thick hyaline membrane. Capillaries appear congested in walls of alveoli and there is infiltration of inflammatory cells. What disease would this most align with?

A

Hyaline Membrane Disease

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11
Q

What are the clinical features of hyaline membrane disease? x5

A
  1. Respiratory distress
  2. Cyanosis
  3. Hypoxemia
  4. Hypercarbia
  5. Respiratory and metabolic acidosis
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12
Q

What are the 2 main complications of Hyaline Membrane Disease?

A

Bronchopulmonary dysplasia
Necrotizing enterocolitis

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13
Q

__________ is a chronic lung disease. Occurs in preterm neonates treated with oxygen therapy > 4 weeks and positive pressure ventilation.

A

Bronchopulmonary dysplasia

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14
Q

_________ leads to reduced total numbers of alveoli, epithelial hyperplasia/squamous metaplasia, and interstitial fibrosis. Sponge-like lung radiology and predisposition to respiratory infection.

A

Bronchopulmonary Dysplasia

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15
Q

What appearance will a lung with Bronchopulmonary Dysplasia have on the external surface?

A

Cobblestone exterior surface due to scarring and alternating hyperinflation and collapse

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16
Q

Necrotizing Enterocolitis is a complication of _________, hyaline membrane disease, and low birth weight

A

Prematurity

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17
Q

What factors contribute to necrotizing enterocolitis?

A

Ischemia
Infection
Enteral feeding

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18
Q

What is the pathogenesis of necrotizing enterocolitis?

A

Ischemia results in focal to confluent areas of bowel necrosis

Most often in terminal ileum, cecum, and right colon

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19
Q

What will the intestine look like with necrotizing enterocolitis?

A

Distended
Congested
Dark red

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20
Q

_________ leads to abdominal distention, ileus, and bloody stool. Gas will appear in the bowel wall. Perforation, strictures, and circulatory collapse may develop.

A

Necrotizing Enterocolitis

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21
Q

What is fetal hydrops?

A

Edema in fetus

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22
Q

What do generalized and localized edema lead to in regards to fetal hydrops?

A

Generalized edema: hydrops fetalis

Localized edema: cystic hygroma

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23
Q

What causes immune hydrops?

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A

Blood group incompatibility

24
Q

What causes non-immune hydrops?

Study These Flashcards
A

Infections
Chromosomal anomalies
Twin pregnancy
Cardiovascular defects

25
With immune hydrops fetalis, a mother with Rh D- and father with Rh D+ have a kid. Fetal RBC may reach maternal circulation during the ________ of pregnancy, when the cytotrophoblast is no longer present as a barrier or __________. This causes maternal immunization to Rh D Ag
last trimester during delivery
26
With immune hydrops fetalis, ______ is produced at the initial exposure to Rh D Ag do not cross the placenta. Thus, immune hydrops fetalis is uncommon with first pregnancy.
Anti-D IgM
27
What is the pathogenesis of immune hydrops fetalis?
Exposure during a subsequent pregnancy leads to brisk anti-D IgG production which can cross the placenta. Transplacental passage of maternal anti-D IgG to fetal Rh D+ RBC
28
Destruction of anti-D IgG-RBC complex with immune hydrops fetalis leads to __________ x2
1. Anemia 2. Hb degradation
29
Immune Hydrops Fetalis leads to hemolytic anemia and hyperbilirubinemia. These lead to _______
Hemolytic anemia - heart failure, liver failure, hydrops fetalis Hyperbilirubinemia - jaundice, kernicterus
30
What does kernicterus present as in gross disection?
Prominent yellow staining of specific region of the brain due to unconjugated bilirubin binds to lipids in brain tissue
31
___________ is unexplained death under 1 years of age after a thorough case investigation, including performance of a complete autopsy, examination of death scene, and review of clinical history.
Sudden Infant Death Syndrome (SIDS)
32
What age group do most cases of SIDS appear in?
90% of cases are where infant is less than 6 months Most are between 2-4 months
33
What are the risk factors for mom of SIDS? x5
1. Young maternal age (<20) 2. Smoking during pregnancy 3. Drug abuse 4. Late or no prenatal care 5. Short intergestational intervals
34
What risk factors for the infant cause SIDS? x5
1. Brain stem abnormality 2. Prematurity/SGA 3. Male 4. Antecedent respiratory infections 5. Multiple birth pregnancy
35
What risk factors for environmental cause SIDS? x4
1. Prone sleep position 2. Sleeping on soft surface 3. Hyperthermia 4. Postnatal passive smoking
36
What is the pathology of SIDS?
Multiple petechiae Lung congestion Hypoplasia of arcuate nucleus and decreased brain stem neuronal populations
37
It can be difficult to distinguish _________ and tumor-like lesions in infant and child.
benign tumors
38
What are examples of developmental vascular anomalies that can be mixed up as benign tumors and tumor like lesions?
Hemangioma Vascular malformation
39
What are examples of lymphatic tumor that can be mixed up as benign tumors and tumor like lesions?
Lymphangioma Lymphangiectasis
40
With fibrous tumors most are ______ except fibrosarcoma.
Benign
41
_______ is also considered a benign tumor and tumor like lesion. Not developmental vascular anomalies, lymphatic tumor, fibrous tumor
Teratoma
42
What is the most common tumor in infants?
Hemangioma
43
What are the two hemangiomas that can occur?
Capillary hemangiomas - strawberry type Cavernous hemangiomas - component of VHL disease
44
Will capillary hemangioma heal with tx?
Will spontaneously regress leaving behind some scarring and deposits of hemosiderin pigment
45
What do you see under the microscope with capillary hemangioma?
Thin walled capillaries with scant stroma
46
What does cavernous hemangioma look like under the microscope?
Large, cavernous blood-filled vascular spaces separated by thick CT stroma
47
What are the 3 categories of fibrous tumors?
1. Infantile myofibromatosis 2. Aggressive infantile fibromatosis 2. Congenital infantile fibrosarcoma
48
____________ can be in congenital infantile fibrosarcoma and it results in production of ETV6-TRKC which is constitutively active and stimulate signaling through the oncogenic RAS and PI-3K/AKT pathways.
Chromosomal translocation t(12;15)(p13;q25)
49
________ is a tumor marker for congenital infantile fibrosarcoma. It is also a marker for secretory breast carcinoma, cellular and mixed types of mesoblastic nephroma and acute myeloid leukemia.
ETV6-TRKC
50
What do we see with infantile myofibromatosis?
Most common fibrous tumor in infants. See multiple myofibromas where cells express muscle specific actin
51
What do we see with aggressive infantile fibromatosis?
Myofibroblast cells infiltrate skeletal muscle. Benign tumor consists fo fibroblasts and myofibroblasts.
52
Does aggressive infantile fibromatosis metastasize?
NO! but it does get large and infiltrate
53
What is the incidence of teratoma?
1/20000-40,000 live births
54
What is a teratoma? Where can they occur?
Germ cell neoplasma Most are sacrococcygeal and mainly in girls Other sites: testis, ovary, mediastinum, retroperitoneum, head, and neck
55
What is the most common solid tumor in a newborn?
Sacrococcygeal Teratoma Most benign!
56
_______ is the most common solid congenital malignancy.
neuroblastoma
57
what is the prognosis for teratomas with age and malignancy?
Age - most benign with young infants (<4 months) Malignancy - occur in 10% of teratomas with immature tissue microscopically

FoundSci III (22 decks)

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