Diseases of Infancy and Childhood - Chapter 10

Question 1

Prematurity is defined as___________________________.

Answer 1

Infants born before completion of the normal gestational period. (p. 456)

Question 2

hat are the leading causes of death in the first twelve months of life?

Answer 2

Congenital anomalies, disorders relating to short gestation (prematurity) and low birth weight, and sudden infant death syndrome. (p. 451)

Question 3

Grossly, immature lungs or lungs from a stillborn infant are ______, _______, and ________.

Answer 3

Unexpanded, red, and meaty (Not in Robbins)

Question 4

What anatomic feature is present in immature kidneys?

Answer 4

Incomplete formation of glomeruli (Not in Robbins)

Question 5

Grossly, the immature liver is _______ relative to the size of the infant and this change is in large part due to ______________________.

Answer 5

Large
Extramedullary hematopoiesis (Not in Robbins)

Question 6

The five clinical signs used in the Apgar scoring of a newborn infant are:

Answer 6

Heart rate, Respiratory effort, muscle tone, response to catheter in nostril, color (Not in Robbins)

Question 7

Birth injuries most commonly involve what parts of the body?

Answer 7

Head, skeletal system, liver, adrenals, and peripheral nerves (Not in Robbins)

Question 8

Define and differentiate Caput Succedaneum and Cephalohematoma.

Answer 8

Caput succedaneum – progressive accumulation of interstitial fluid in the soft tissues of the scalp, giving rise to a usually circular area of edema, congestion, and swelling at the site where the head begins to enter the lower uterine wall.

Cephalohematoma – hemorrhage occurring in the scalp. (Not in Robbins)

Question 9

What is the most common, important pathologic finding associated with birth head injuries?

Answer 9

Intracranial hemorrhage (Not in Robbins)

Question 10

The most common birth injuries of the skeletal system are:

Answer 10

Clavicular and humeral fractures (Not in Robbins)

Question 11

Anomalies known to be genetic in origin can be divided into what two groups?

Answer 11

Those associated with chromosomal aberrations
Those arising from single gene mutations (p. 454)

Question 12

List four of the most common chromosomal syndromes.

Answer 12

Trisomy 21, Klinefelter syndrome, Turner syndrome, Trisomy 13 (Not in Robbins)

Question 13

List three of the most common environmental influences that produce malformations in the fetus and infant.

Answer 13

Viral infections, drugs, and irradiation (p. 454)

Question 14

The two most investigated viral infections of the newborn are:

Answer 14

Rubella virus and cytomegalovirus (p. 454)

Question 15

The major clinical tetrad seen the “congenital rubella syndrome” is composed of _____, ______, and ______.

Answer 15

Cataracts, heart defects, deafness, and mental retardation (Not in Robbins)

Question 16

The most common cause of neonatal respiratory distress syndrome is also known as ______and is most commonly associated with________.

Answer 16

Hyaline membrane disease Prematurity (p. 457)

Question 17

The fundamental defect in respiratory distress syndrome is __________.

Answer 17

Deficiency of pulmonary surfactant (p. 457)

Question 18

Grossly, lungs from a patient with respiratory distress syndrome are__________.

Answer 18

Solid, airless, and reddish purple similar to the color of liver, and they usually sink in water. (p. 458)

Question 19

he primary therapeutic modality in respiratory distress syndrome is______ which, when administered for prolonged period of time, may cause __________.

Answer 19

The delivery of surfactant replacement therapy and oxygen

Retrolental fibroplasia in the eyes and bronchopulmonary dysplasia (p. 459)

Question 20

The disease seen as a result of prolonged oxygen toxicity in hyaline membrane disease is______.

Answer 20

Bronchopulmonary dysplasia (p. 459)

Question 21

Define immune hydrops. What pathologic process occurs to allow the development of immune

Answer 21

Immune hydrops is a hemolytic disease caused by blood group incompatibility between mother and fetus. The underlying basis of immune hydrops is the immunization of the mother by blood group antigens on fetal red cells and the free passage of antibodies from the mother through the placenta to the fetus. (p. 461)

Question 22

The most common lethal genetic disease that affects the Caucasian population is ______.

Answer 22

Cystic fibrosis (p. 464)

Question 23

What are the two consequences of excessive destruction of red blood cells in the neonate?

Answer 23

Anemia and jaundice (p. 462)

Question 24

The most serious problem in erythroblastosis fetalis is damage to the ________ also known as_______.

Answer 24

Nervous system Kernicterus (p. 462-463

Question 25

The most common histologic changes in erythroblastosis fetalis are:

Answer 25

Identification of abnormally increased erythropoietic activity, increased numbers of reticulocytes, normoblasts, and erythroblasts in the circulating blood. (p. 462)

Question 26

Define cystic fibrosis, and include a synonymous term.

Answer 26

Cystic fibrosis (mucoviscidosis) is a disorder of ion transport in epithelial cells affecting fluid secretion in exocrine glands and the epithelial lining of the respiratory, gastrointestinal, and reproductive tracts. This disorder leads to abnormally viscid mucous secretions, which obstruct organ passages. (p. 466)

Question 27

Briefly describe the pathologic changes in the pancreas of patients with cystic fibrosis.

Answer 27

mild - accumulations of mucous in the small ducts with some dilation of the exocrine glands. Advanced stage – ducts are completely plugged, causing atrophy of the exocrine glands and progressive fibrosis.

Question 28

Briefly describe the pathologic changes in the liver of patients with cystic fibrosis.

Answer 28

Bile canaliculi are plugged by mucinous material accompanied by ductular proliferation and portal inflammation. Biliary cirrhosis may develop.

Question 29

Briefly describe the pathologic changes in the Salivary glands of patients with cystic fibrosis.

Answer 29

Salivary glands – progressive dilation of ducts, squamous metaplasia of the lining epithelium,
and glandular atrophy followed by fibrosis.

Question 30

Briefly describe the pathologic changes in the lungs of patients with cystic fibrosis.

Answer 30

Lungs – viscous mucus secretions of the submucosal glands of the respiratory tree with secondary obstruction and infection of the air passages. The bronchioles are often distended with thick mucus associated with marked hyperplasia and hypertrophy of the mucus-secreting cells.
(p. 469-470)

Question 31

Define sudden infant death syndrome (SIDS).

Answer 31

The sudden death of an infant under one year of age, which remains unexplained after a thorough case investigation, including performance of a complete autopsy, examination of the death scene, and review of the clinical history. (p. 471)

Question 32

What is the usual clinical presentation of SIDS?

Answer 32

Infants die at home, usually during the night after a period of sleep, 90% during the first 6 months of life. (p. 471)

Question 33

he most common pathologic findings in infants who have died of suspected SIDS are:

Answer 33

Multiple petechiae – thymus, visceral and parietal pleura, and epicardium (p. 472)

Question 34

Define choristoma and give an example

Answer 34

Choristoma or heterotopia is applied to microscopically normal cells or tissues that are present inabnormal locations. (pancreatic rest in wall of stomach or small intestine)

Question 35

Define hamartoma and give an example

Answer 35

Hamartoma refers to an excessive but focal overgrowth of cells and tissues native to the organ in which it occurs.
(pulmonary hamartoma of mature cartilage) (p. 473

Question 36

the most common tumor of infancy is __________.

Answer 36

Hemangioma (p. 474)

Question 37

Name eight of the most common malignant neoplasms seen in children under five years of age.

Answer 37

Leukemia
Retinoblastoma
Neuroblastoma
Wilms tumor
Hepatoblastoma
soft tissue sarcoma, teratoma
CNS tumors (p. 475)

Question 38

What is the most common extracranial tumor of childhood?

Answer 38

Neuroblastoma (p. 475)

Question 39

List three clinical syndromes associated with an increased incidence of Wilms tumor.

Answer 39

WAGR syndrome
Denys-Drash syndrome
Beckwith-Wiedemann syndrome (p. 479-480)

Question 40

What primary error of morphogenesis occurs in which there is an intrinsically abnormal development process (multifactorial)?

Answer 40

Malformation (p. 452)

Question 41

What error of morphogenesis results from secondary destruction of an organ or body region that was previously normal in development?

Answer 41

Disruption (p. 452) ( fix this looks wrong)

Question 42

What is a cascade of anomalies triggered by one initiating aberration?

Answer 42

Sequence (p. 452) also seems wrong

Question 43

What is a constellation of congenital anomalies believed to be pathologically related and cannot be explained on the basis of a single localized, initiating defect?

Answer 43

Syndrome (p. 453) what fix this

Question 44

What is a constellation of congenital anomalies believed to be pathologically related and cannot be explained on the basis of a single localized, initiating defect?

Answer 44

Syndrome (p. 453)