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

Chapter 10- Diseases Of Infancy And Childhood Flashcards

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
Q

When is an embryo/fetus most susceptible to teratogens?

A

3-9wks

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26
Q

What is the embryonic period of gestation?

A

1-8wks

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27
Q

At what week of gestation are newborns considered premature?

A

Earlier than 37 weeks

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28
Q

What is the difference between preterm premature rupture of placental membrane (PPROM) and PROM?

A

PPROM- occurs before 37wks, higher risk for mortality

PROM- after 37wks, less risk associated

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29
Q

What factors can predispose a pregnancy to PPROM?

A

Prior hx

Smoking

Vaginal bleeding

Poor nutrition

Low socioeconomic status

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30
Q

How common is intrauterine infection?

A

Occurs in 25% of preterm births

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31
Q

How do intrauterine infections cause preterm birth?

A

TLR activation down-regulates prostaglandin, inducing smooth muscle contraction

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32
Q

What is chorioamnionitis?

A

Placental membrane inflammation

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33
Q

What is funisitis?

A

Umbilical cord inflammation

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34
Q

What organisms are associated with intrauterine infections?

A

Ureaplasma urealyticum

M. hominis

G. vaginalis

Trichomonas

Gonorrhea

Chlamydia

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35
Q

What types of structural abnormalities can cause premature birth?

A

Fibroids (leiomas)

Placenta previa- cervix is blocked by the placenta

Abruptio placentae- premature separation between the placenta and uterus

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36
Q

What are the hazards associated with premature birth?

A

Neonatal respiratory distress syndrome/hyaline membrane disease

Necrotizing enterocolitis

Sepsis

Intraventricular and germinal matrix hemorrhage

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37
Q

What is intraventricular and germinal matrix hemorrhage?

A

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

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38
Q

What can cause premature birth?

A

PPROM

Intrauterine infection

Gestation with multiples

Uterine, cervical and placental structural abnormalities

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39
Q

What birth weight is indicative of fetal growth restriction?

A

<2500g full term

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40
Q

What kind of anomalies can cause fetal growth restriction?

A

Fetal

Placental

Maternal

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41
Q

Fetal anomalies causing growth restriction are normally what?

A

Symmetric

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42
Q

What fetal anomalies can cause growth restriction?

A

Chromosome disorders

Congenital anomalies

Fetal infection (TORCH)

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43
Q

What placental anomalies can cause fetal growth restriction?

A

Uteroplacental insufficiency

Umbilical placental vascular anomalies

Placental abruptio

Placenta previa

Placental thrombosis and infarction

Placental infection

Pregnancy with multiples

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44
Q

What do most placental abnormalities causing fetal growth restriction stem from?

A

Heavy demands on blood supply during the third trimester

45
Q

What is the most common factor in maternal anomalies causing fetal growth restriction?

A

Conditions reducing placental blood flow

46
Q

What maternal abnormalities can cause fetal growth restriction?

A

Pre-eclampsia

Chronic hypertension

Narcotics, alcohol, smoking, malnutrition

47
Q

What is the cause of neonatal respiratory distress syndrome (NRDS)?

A

Lung immaturity

Deficiency in pulmonary surfactant secreted by Type II pneumocytes

Alveoli are difficult to fill, collapse on themselves

48
Q

NRDS is less common with what?

A

Increasing age

49
Q

When does surfactant production begin in utero?

A

35wks

50
Q

What stimulates surfactant production?

A

Corticosteroids

51
Q

What do the lungs of a NRDS neonate look like?

A

Solid, airless, reddish-purple

Sink in water

52
Q

What is hyaline membrane disease?

A

Thick hyaline membranes line the alveoli

53
Q

What are NRDS neonates at an increased risk of developing?

A

Patent ductus arteriosus (increased lung BP- more blood)

Intraventricular hemorrhage

Necrotizing enterocolitis

54
Q

What is necrotizing enterocolitis?

A

Ischemic necrosis of the intestinal mucosa in portions d the bowel

55
Q

What is necrotizing enterocolitis inversely proportional to?

A

Age

56
Q

What are the characteristics of necrotizing enterocolitis?

A

Distended, friable, congested or frankly gangrenous bowel

Intestinal perforation with accompanying peritonitis

Pneumatosis intestinalis- gas within intestinal wall

57
Q

What is the microscopic picture of necrotizing enterocolitis?

A

Coagulative necrosis

Ulceration

Colonization

Submucosal gas bubbles

58
Q

How do perinatal infections occur?

A

Transvertically/ascending- amniotic fluid, birth canal

Transplacentally/hematologically- parasites, viruses, listeria and treponema

59
Q

What is fetal hydrops?

A

Accumulation of edema fluid

60
Q

What are the types of fetal hydrops?

A

Hydrops fetalis (generalized)

Cystic hygroma

Immune hydrops

Non-immune hydrops

61
Q

Where does cystic hygroma occur?

A

Post-nuchally (behind neck)

62
Q

What is immune hydrops?

A

Hemolytic disease caused by blood group Ag incompatibility

63
Q

What Ag group normally causes immune hydrops?

A

Rh

64
Q

Why does ABO rarely cause immune hydrops?

A

IgM (can’t cross placenta)

Ags are poorly expressed on fetal cell

65
Q

What are the characteristics of immune hydrops?

A

Anemia (hemolysis)

Heart and liver damage

Jaundice

Kernicterus

66
Q

Why does jaundice occur in immune hydrops?

A

Fetus lacks enzyme to conjugate bilirubin, unconjugated bilirubin from hemolysis builds up

67
Q

What is kernicterus?

A

Bilirubin binds lipids in the brain causing retardation

68
Q

What are the major causes of non-immune hydrops?

A

CV defects

Chromosomal anomalies (Turner syndrome- 45X)

Fetal anemia- pale fetus

69
Q

How does twin to twin transfusion cause non-immune hydrops?

A

Blood is shunted from one to another (one has almost none, the other has too much)

70
Q

What infection can cause non-immune hydrops?

A

Parvo B19

71
Q

What does non-immune hydrops caused by Turner syndrome look like?

A

Abnormalities of lymph fluid drainage from the neck

72
Q

What are common inborn errors of metabolism?

A

Phenylketonuria

Galactosemia

Cystic fibrosis

73
Q

What is deficient in phenylketonuria?

A

Phenylalanine hydroxylase

74
Q

What are the characteristics of phenylketonuria?

A

Mental retardation

Reduced hair and skin pigmentation

Musty/mousy odour

Build up of phenylalanine from mother’s diet

75
Q

What screen is used for phenylketonuria?

A

PKU

76
Q

How is phenylketonuria treated?

A

Restrictive diets

77
Q

What is deficient in galactosemia?

A

Enzyme that metabolizes galactose

78
Q

What are the characteristics of galactosemia?

A

Build up of galactose-1-phosphate

Hepatomegaly

Cataracts

CNS alterations

Failure to thrive

Vomiting and diarrhea after milk ingestion

79
Q

How is galactosemia treated?

A

Restrictive diets

80
Q

What is cystic fibrosis?

A

Inherited disorder if ion transport that affects fluid secretion

81
Q

What is the most common lethal genetic disease in caucasians?

A

Cystic fibrosis

82
Q

What is mutated in cystic fibrosis?

A

CF transmembrane conductance regulating protein (CFTR)

83
Q

What ion does the CFTR transport?

A

Chloride (other membrane transports salt, water follows)

84
Q

What abnormalities are seen with cystic fibrosis?

A

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
Q

What is the definitive test for cystic fibrosis?

A

Sweat chlorides

86
Q

What is the major cause of death in cystic fibrosis?

A

Cardiorespiratory disease

87
Q

What are the cardiorespiratory diseases associated with cystic fibrosis?

A

Lung infections

Obstructive lung disease

Cor pulmonale

88
Q

What is SIDS?

A

Sudden death of an infant that can’t be explained after investigation

89
Q

What is sudden unexpected infant death syndrome?

A

Cause is discovered during autopsy

90
Q

What is the morphology of SIDS?

A

Multiple petechiae

Evidence of upper respiratory tract infection

Astrogliosis of brain and cerebellum

91
Q

What is the suggested pathogenesis of SIDS?

A

Immature critical brain stem regions and environmental influences impair regulatory mechanisms

92
Q

What risk factors are associated with SIDS?

A

Maternal age and health

Brain stem abnormalities

Prematurity

Birth of multiples

SIDS in previous sibling

Prone or side sleeping

Soft sleeping surface

93
Q

What are the most common neoplasms of infancy?

A

Mesenchymal (sarcomas)

94
Q

What is the most common tumour of infancy?

A

Hemangioma

95
Q

What are hemangiomas sometimes associated with?

A

von Hippel-Lindau disease

96
Q

What are the characteristics of hemangiomas?

A

Vascular tumours

Irregular red-blue masses

Port wine stains

97
Q

When are lymphatic tumours clinically significant in infants?

A

When they encroach on viral structures

98
Q

What fibrous tumour has an excellent prognosis?

A

Infantile fibrosarcoma (ETV6-NTRK3 fusion gene)

99
Q

When are the peak incidences of teratomas in children?

A

2yrs and late adolescence

100
Q

Where do 40% of teratomas occur in children?

A

Sacrococcygeal region

101
Q

What cancer is responsible for the most deaths in children?

A

Leukemias

102
Q

What are the common forms of malignant tumours in children?

A

Small round blue cell tumours

-blastomas

103
Q

Where do neuroblastic tumours occur?

A

Medulla or sympathetic ganglion

104
Q

What is the most common neuroblastic tumour?

A

Neuroblastoma

105
Q

What are the characteristics of neuroblastomas?

A

Neurofibrillary background

Homer Wright pseudorosettes

106
Q

What are Wilm’s tumours?

A

Soft, large, well circumscribed renal masses

107
Q

What is the triphasic histology of Wilm’s tumours?

A

Blastema

Immature stroma

Tubules

108
Q

What mutation is associated with Wilm’s tumours?

A

Bilateral germline mutations in 11p

109
Q

What syndromes are associated with Wilm’s tumours and what are their characteristics?

A

WAGR- anirida, genital anomalies, retardation

Denys-Drash- gonadal dysgenesis, nephropathy

Beckwith-Wiedemann- enlarged organs, hemihypertrophy

Pathology (25 decks)

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