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Pathology > Chapter 10- Diseases Of Infancy And Childhood > Flashcards

Chapter 10- Diseases Of Infancy And Childhood Flashcards

(109 cards)

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1
Q

What are the stages of development?

A

Neonatal- first 4wks

Infancy- first year

1-4

5-14

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2
Q

What are the most prominent disorders associated with each developmental stage?

A

Infant- congenital disorders

1-4- accidents

5-9- accidents with neoplasms becoming more prominent

10-14- accidents with suicides becoming more prominent

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3
Q

What are congenital anomalies?

A

Defects present at birth

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4
Q

What is the most common cause of mortality in infants?

A

Congenital anomalies

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5
Q

What is blighted ovum/anembryonic pregnancy?

A

No embryo/gestational sac produced

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6
Q

When is miscarriage most common?

A

First 8wks

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7
Q

What are malformations?

A

Primary errors of morphogenesis due to abnormal development

Genetic

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8
Q

What are disruptions?

A

Secondary organ/body region destruction

Extrinsic/environmental

Structure was previously normal

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9
Q

What are deformations?

A

Localized/generalized compression of the fetus by abnormal biochemical factors

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10
Q

What commonly causes disruptions?

A

Amniotic bands

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11
Q

What can cause deformations?

A

Uterine constraint

Multiple fetuses

Bicornate uterus

Leiomyomas

Oligohydroamnios

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12
Q

What is a sequence?

A

A cascade of anomalies triggered by one event

Single localized aberration in organogenesis sets secondary effects involving other organs into motion

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13
Q

What is a common sequence?

A

Potter sequence/oligohydroamnios

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14
Q

What are the characteristics of Potter sequence?

A

Reduced amniotic fluid (multiple possible causes) results in flattened face, positional abnormalities, pulmonary hypoplasia

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15
Q

What is a malformation syndrome?

A

A constellation of congenital anomalies that can’t be explained by a single initiating event

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16
Q

What is agenesis?

A

Organ absence (also associated primordium)

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17
Q

What is aplasia?

A

Organ absence due to failure of growth of the existing primordium

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18
Q

What is atresia?

A

Absence of an opening

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19
Q

Where is atresia normally seen?

A

Hollow organs

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20
Q

What is dysplasia (in disorders of infancy)?

A

Abnormal organization of cells

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21
Q

What are possible causes of dysplasia in development?

A

Genetic- chromosomal disorders associated with congenital malformations

Environmental

Multifactorial- environment influences and two or more genes of small effect

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22
Q

What environmental factors can cause congenital disorders?

A

Viral infections

Drugs

DM

Irradiation

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23
Q

What does TORCH stand for? And what do these infections often cause?

A

Congenital defects in utero

Toxoplasmosis

Other (syphilis, VZ, parvo)

Rubella

CMV

Herpes

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24
Q

What congenital disorders are commonly multifactorial?

A

Cleft lip/palate

Neural tube defects

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25
When is an embryo/fetus most susceptible to teratogens?
3-9wks
26
What is the embryonic period of gestation?
1-8wks
27
At what week of gestation are newborns considered premature?
Earlier than 37 weeks
28
What is the difference between preterm premature rupture of placental membrane (PPROM) and PROM?
PPROM- occurs before 37wks, higher risk for mortality PROM- after 37wks, less risk associated
29
What factors can predispose a pregnancy to PPROM?
Prior hx Smoking Vaginal bleeding Poor nutrition Low socioeconomic status
30
How common is intrauterine infection?
Occurs in 25% of preterm births
31
How do intrauterine infections cause preterm birth?
TLR activation down-regulates prostaglandin, inducing smooth muscle contraction
32
What is chorioamnionitis?
Placental membrane inflammation
33
What is funisitis?
Umbilical cord inflammation
34
What organisms are associated with intrauterine infections?
Ureaplasma urealyticum M. hominis G. vaginalis Trichomonas Gonorrhea Chlamydia
35
What types of structural abnormalities can cause premature birth?
Fibroids (leiomas) Placenta previa- cervix is blocked by the placenta Abruptio placentae- premature separation between the placenta and uterus
36
What are the hazards associated with premature birth?
Neonatal respiratory distress syndrome/hyaline membrane disease Necrotizing enterocolitis Sepsis Intraventricular and germinal matrix hemorrhage
37
What is intraventricular and germinal matrix hemorrhage?
Lesion, normally starting between thalamus and caudate nucleus Large- rupture into ventricles (germinal matrix is immature cells under their lining) Morbidity, cerebral palsy, retardation Bilateral
38
What can cause premature birth?
PPROM Intrauterine infection Gestation with multiples Uterine, cervical and placental structural abnormalities
39
What birth weight is indicative of fetal growth restriction?
<2500g full term
40
What kind of anomalies can cause fetal growth restriction?
Fetal Placental Maternal
41
Fetal anomalies causing growth restriction are normally what?
Symmetric
42
What fetal anomalies can cause growth restriction?
Chromosome disorders Congenital anomalies Fetal infection (TORCH)
43
What placental anomalies can cause fetal growth restriction?
Uteroplacental insufficiency Umbilical placental vascular anomalies Placental abruptio Placenta previa Placental thrombosis and infarction Placental infection Pregnancy with multiples
44
What do most placental abnormalities causing fetal growth restriction stem from?
Heavy demands on blood supply during the third trimester
45
What is the most common factor in maternal anomalies causing fetal growth restriction?
Conditions reducing placental blood flow
46
What maternal abnormalities can cause fetal growth restriction?
Pre-eclampsia Chronic hypertension Narcotics, alcohol, smoking, malnutrition
47
What is the cause of neonatal respiratory distress syndrome (NRDS)?
Lung immaturity Deficiency in pulmonary surfactant secreted by Type II pneumocytes Alveoli are difficult to fill, collapse on themselves
48
NRDS is less common with what?
Increasing age
49
When does surfactant production begin in utero?
35wks
50
What stimulates surfactant production?
Corticosteroids
51
What do the lungs of a NRDS neonate look like?
Solid, airless, reddish-purple Sink in water
52
What is hyaline membrane disease?
Thick hyaline membranes line the alveoli
53
What are NRDS neonates at an increased risk of developing?
Patent ductus arteriosus (increased lung BP- more blood) Intraventricular hemorrhage Necrotizing enterocolitis
54
What is necrotizing enterocolitis?
Ischemic necrosis of the intestinal mucosa in portions d the bowel
55
What is necrotizing enterocolitis inversely proportional to?
Age
56
What are the characteristics of necrotizing enterocolitis?
Distended, friable, congested or frankly gangrenous bowel Intestinal perforation with accompanying peritonitis Pneumatosis intestinalis- gas within intestinal wall
57
What is the microscopic picture of necrotizing enterocolitis?
Coagulative necrosis Ulceration Colonization Submucosal gas bubbles
58
How do perinatal infections occur?
Transvertically/ascending- amniotic fluid, birth canal Transplacentally/hematologically- parasites, viruses, listeria and treponema
59
What is fetal hydrops?
Accumulation of edema fluid
60
What are the types of fetal hydrops?
Hydrops fetalis (generalized) Cystic hygroma Immune hydrops Non-immune hydrops
61
Where does cystic hygroma occur?
Post-nuchally (behind neck)
62
What is immune hydrops?
Hemolytic disease caused by blood group Ag incompatibility
63
What Ag group normally causes immune hydrops?
Rh
64
Why does ABO rarely cause immune hydrops?
IgM (can’t cross placenta) Ags are poorly expressed on fetal cell
65
What are the characteristics of immune hydrops?
Anemia (hemolysis) Heart and liver damage Jaundice Kernicterus
66
Why does jaundice occur in immune hydrops?
Fetus lacks enzyme to conjugate bilirubin, unconjugated bilirubin from hemolysis builds up
67
What is kernicterus?
Bilirubin binds lipids in the brain causing retardation
68
What are the major causes of non-immune hydrops?
CV defects Chromosomal anomalies (Turner syndrome- 45X) Fetal anemia- pale fetus
69
How does twin to twin transfusion cause non-immune hydrops?
Blood is shunted from one to another (one has almost none, the other has too much)
70
What infection can cause non-immune hydrops?
Parvo B19
71
What does non-immune hydrops caused by Turner syndrome look like?
Abnormalities of lymph fluid drainage from the neck
72
What are common inborn errors of metabolism?
Phenylketonuria Galactosemia Cystic fibrosis
73
What is deficient in phenylketonuria?
Phenylalanine hydroxylase
74
What are the characteristics of phenylketonuria?
Mental retardation Reduced hair and skin pigmentation Musty/mousy odour Build up of phenylalanine from mother’s diet
75
What screen is used for phenylketonuria?
PKU
76
How is phenylketonuria treated?
Restrictive diets
77
What is deficient in galactosemia?
Enzyme that metabolizes galactose
78
What are the characteristics of galactosemia?
Build up of galactose-1-phosphate Hepatomegaly Cataracts CNS alterations Failure to thrive Vomiting and diarrhea after milk ingestion
79
How is galactosemia treated?
Restrictive diets
80
What is cystic fibrosis?
Inherited disorder if ion transport that affects fluid secretion
81
What is the most common lethal genetic disease in caucasians?
Cystic fibrosis
82
What is mutated in cystic fibrosis?
CF transmembrane conductance regulating protein (CFTR)
83
What ion does the CFTR transport?
Chloride (other membrane transports salt, water follows)
84
What abnormalities are seen with cystic fibrosis?
Pancreas- mucous accumulation Intestine- meconium ileus (mucous plugs) Liver- cirrhosis (mucin plugs) Salivary glands- duct dilation, atrophy, squamous metaplasia Lungs- mucous cell hyperplasia, viscous secretions block/dilate bronchioles Male genital tract- azoospermia, infertility Cardiorespiratory disease Sweat glands- extremely concentrated excretions
85
What is the definitive test for cystic fibrosis?
Sweat chlorides
86
What is the major cause of death in cystic fibrosis?
Cardiorespiratory disease
87
What are the cardiorespiratory diseases associated with cystic fibrosis?
Lung infections Obstructive lung disease Cor pulmonale
88
What is SIDS?
Sudden death of an infant that can’t be explained after investigation
89
What is sudden unexpected infant death syndrome?
Cause is discovered during autopsy
90
What is the morphology of SIDS?
Multiple petechiae Evidence of upper respiratory tract infection Astrogliosis of brain and cerebellum
91
What is the suggested pathogenesis of SIDS?
Immature critical brain stem regions and environmental influences impair regulatory mechanisms
92
What risk factors are associated with SIDS?
Maternal age and health Brain stem abnormalities Prematurity Birth of multiples SIDS in previous sibling Prone or side sleeping Soft sleeping surface
93
What are the most common neoplasms of infancy?
Mesenchymal (sarcomas)
94
What is the most common tumour of infancy?
Hemangioma
95
What are hemangiomas sometimes associated with?
von Hippel-Lindau disease
96
What are the characteristics of hemangiomas?
Vascular tumours Irregular red-blue masses Port wine stains
97
When are lymphatic tumours clinically significant in infants?
When they encroach on viral structures
98
What fibrous tumour has an excellent prognosis?
Infantile fibrosarcoma (ETV6-NTRK3 fusion gene)
99
When are the peak incidences of teratomas in children?
2yrs and late adolescence
100
Where do 40% of teratomas occur in children?
Sacrococcygeal region
101
What cancer is responsible for the most deaths in children?
Leukemias
102
What are the common forms of malignant tumours in children?
Small round blue cell tumours -blastomas
103
Where do neuroblastic tumours occur?
Medulla or sympathetic ganglion
104
What is the most common neuroblastic tumour?
Neuroblastoma
105
What are the characteristics of neuroblastomas?
Neurofibrillary background Homer Wright pseudorosettes
106
What are Wilm’s tumours?
Soft, large, well circumscribed renal masses
107
What is the triphasic histology of Wilm’s tumours?
Blastema Immature stroma Tubules
108
What mutation is associated with Wilm’s tumours?
Bilateral germline mutations in 11p
109
What syndromes are associated with Wilm’s tumours and what are their characteristics?
WAGR- anirida, genital anomalies, retardation Denys-Drash- gonadal dysgenesis, nephropathy Beckwith-Wiedemann- enlarged organs, hemihypertrophy

Pathology (25 decks)

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