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Pathology > Pediatrics > Flashcards

Pediatrics Flashcards

1
Q

Types of Congenital Anomalies

A 
Malformations
Disruptions
Deformations
Sequences
Syndromes
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2
Q

Primary errors of morphogenesis

Intrinsically abnormal developmental process

A

Malformations

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3
Q

Anencephaly
Arnold-Chiari Malformation

Are examples of

A

Primary errors of morphogenesis

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4
Q

Associated with folic acid deficiency

A

Anencephaly

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5
Q

Extrinsic disturbances of development

Due to localized or generalized compression

A

Deformation

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6
Q

Most common cause of deformation

A

Uterine constraint

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7
Q

Example of deformation

A

Club foot talipes equinovarus

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8
Q

Cascade of anomalies triggered by one initiating aberration

A

Sequences

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9
Q

Sequence by oligohydramnios

A

Potter sequence

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10
Q

Sequence induced with the use of misoprostol (PGE?)

Facial paralysis with inability to move eyes from side to side

A

Mobius sequence

PGE2 agonist
Abortifacient - unwanted uterine contractions

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11
Q

Oligohydramnios can induce

A

Fetal renal agenesis
Amniotic leak

Fetal compression
Pulmonary hypoplasia
Altered facies
Positioning defects of feets and hands
Breech presentation
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12
Q

Renal agenesis is associated with the drug

A

ACE I

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13
Q

B Renal Agenesis
B Multicystic Dysplastic Kidneys
Reduced Fetal urine excretion
Oligohydramnios causing fetal compression:

Low set ears
Beaked bose
Twisted skin and face
Prominent epicanthic folds and downward slant to eyes
Pulmonary hyooplasia causing respiratory failure
Limb deformities

A

Potter’s sequence

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14
Q

Constellation of congenital anomalies

Cannot be explained on the basis of a single defect

Ex: Congenital rubella syndrome

A

Syndromes

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15
Q 
Intrauterine Growth Retardation
Congenital cataracts (1/3 of patients)
Microcephaly
Structural heard defects like PDA
Salt-and-pepper retinopatht
Blueberry muffin skin lesions
A

Congenital rubella syndrome

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16
Q

Most common manifestation of CRS

A

Sensorineural hearing loss

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17
Q

Measles or Rubella in first trimester is associated

A

with more severe anomaly

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18
Q

Leukocoria

Cat’s eye

A

Retinoblastoma

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19
Q

Prematurity induces

formation of hyaline membrane disease

A 
Reduced surfactant synthesis, storage, release
Decreased alveolar surfactant*
Increased alveolar surface tension
Atelectasis (uneven perfusion, hypoventilation)*
Hypoxemia + CO2 retention *
Acidosis
Pulmonary vasoconstriction
Pulmonary hypoperfusion
Endothelial damage*
Epithelial damage*
Increased diffusion gradient 
HMD*
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20
Q

Solid
Airless lungs
Sink in water
Necrotic cellular debris

A

Hyaline membrane disease

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21
Q

Which test determines whether respiration took place on a newborn before death?

A

Fodere’s test

Hydrostatic test

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22
Q

Which test involves floating of the stomach in water to determine the presence of air?

A

Breslau’s test

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23
Q

Abusive head trauma

Retinal hemorrhage
Brain swelling
Subdural hematoma

A

Shaken baby syndrome

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24
Q

Sudden and unexpected death of an infant before 1 year of age whose death remains unexplained after autopsy

Most common cause of death in less than 1

Most occur between 2 and 4 months of age

A

Sudden Infant Death Syndrome

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25
Q

Most common finding in SIDS

A

Multiple petechiae

Others:
vascular engorgement
pulmonary edema
astrogliosis of brain stem and cerebellum

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26
Q

Hypoplasia of arcuate nucleus
Persistence of hepatic extramedullary hematopoiesis
Persistence of periadrenal brown fat

A

Sudden Infant Death Syndrome

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27
Q

What is the only safe sleeping position for infants that reduces the incidence of sudden infant death syndrome?

A

Supine position

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28
Q

Complications of oxygen therapy

A

Retrolental fibroplasia
(retinopathy of prematurity)
VEGF causes retinal neurovascularization

Bronchopulmonary dysplasia

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29
Q

VEGF is also called

A

Vascular permeability factor

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30
Q

Decrease in alveolar septation
Fibrosis
Dysmorphic capillary configuration

due to immature antioxidant defenses

A

Bronchopulmonary dysplasia

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31
Q

Breakdown of mucosal barrier functions permits transluminal migration of bacteria

Most common surgical emergency in neonate

A

Necrotizing enterocolitis

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32
Q

Most important risk factor for N.E.C.

A

Prematurity

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33
Q

Bloody stool
Abdominal distention
Development of circulatory collapse

A

Necrotizing enterocolitis

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34
Q

Gas within intestinal walls

A

pneumatosis intestinalis

35
Q

by hemolytic disease caused by blood group incompatibility

A

Immune hydrops

36
Q

May also be called by Parvovirus B19 infection or Twin-twin transfusion syndrome

A

Non-immune hydrops

37
Q

Rh - mother previously sensitized to Rh antigen by transfusion or Rh+ fetus

Stimulate antibody production against Rh antigen

IgG crosses placenta with antibody attachment to Rh+ erythrocytes of fetus

Removal and destruction of erythrocyte-antibody complex

Anemia, Hemoglobin degradation

Extramedullary hematopoiesis, Cardiac decompensation

Bilirubin increase, jaundice

A

Hydrops fetalis

38
Q

Immune hydrops prevention

A

Administration of RhIg to Rh negative mothers

at 28 weeks AOG, within 72h of delivery or following abortions

39
Q

Compensatory hyperplasia of bone marrow

results in

A

Hydrops fetalis

Erythroblastosis fetalis
large numbers of rbcs

40
Q 
Kernicterus
Hemolytic anemia
Jaundice
Chorea
Athetosis
A

Erythroblastosis fetalis

41
Q

Kericterus has special predilection to

A 
basal ganglia
thalamus
cerebellum
cerebral gray matter
SC
42
Q

Bilirubin deposits in basal ganglia of kernicterus baby is

A

> 20 mg/dl

43
Q

Autosomal recessive

Mutations of gene encoding phenylalanine hydroxylase

Inability to convert phenylalanine into tyrosine

Excess phenylalanine metabolites cause brain damage and mental retardation

A

Phenylketonuria

44
Q

Imparts a strong musty or mousy odor to affected infants

A

Phenylacetic acid

45
Q

Galactose-1-phosphate uridyl transferase

Galactokinase

A

Galactosemia

46
Q

Precursor of tyrosine

Rate limiting enzyme?

Cofactor?

A

Phenylalanine

Phenylalanine hydroxylase

Tetrahydrobiopterine

47
Q

Most common galactosemia variant

A

Galactose 1-phosphate uridyl transferase

48
Q 
E coli septicemia
Hepatomegaly
Cataract
Failure to thrive
Mental retardation
A

Galactosemia

49
Q

Tx for PKU

A

Sapropterin Dihydrochloride (Tetrahydrobiopterin BH4 THB)

to decrease phenylalanine

50
Q

Disorder of ion transport in epithelial cells
Autosomal recessive transmission
Most common lethal genetic disease that affects Caucasian populations

A

Cystic fibrosis

51
Q

Deletion of three nucleotides coding for phenylalanine at amino acid position 508 (delta F508)

Production of defective CFTR for chloride

Loss of CFTR causes decreased Na and Cl reabsorption in sweat glands

Basis of sweat chloride test

A

Cystic fibrosis

52
Q

Atrophy of exocrine pancreas
Meconium ileus (thick viscid mucus plugs causing SBO in infants)
Respiratory infections
Infertility (azoospermia, bilateral absence of vas deferens)

A

Cystic fibrosis

53
Q

Cataract is galactosemia is caused by accumulation of

In DM?

A

Galactitol in lens
Oil drop cataract

Sorbitol

54
Q

Respiratory infections in cystic fibrosis are most commonly due to

A

Pseudomonas aeruginosa

55
Q

Gene erroneous in cystic fibrosis

A

CFTR Gene

Cystic fibrosis transmembrane regulator gene

56
Q

Most common lethal genetic disease in Caucasians

A

Cystic fibrosis

57
Q

Most common vascular tumors of infancy

Flat larger lesions (port-wine stain)

A

Hemangiomas

58
Q

Most common teratomas of childhood

Germ cell tumor

A

Sacrococcygeal teratoma

Composed of developed tissues/organs

Hair
Teeth
Muscle
Bone

59
Q

Forms of teratoma

A

Mature
Immature
Unequivocally malignant

60
Q

Tumors of sympathetic ganglia and adrenal medulla
Derived from primodial neural crest cells
Amplification of N-MYC oncogene

Peak age at 18 months

A

Neuroblastoma

61
Q

Most common extracranial solid tumor of childhood

A

Neuroblastoma

62
Q

Gene amplification of

Neuroblastoma

A

N-MYC oncogene

63
Q

Multipel fluid loops of intestine

Meconium ileus on XRAY

A

Soap bubble sign

64
Q

Meconeum ileus is associated to

A

Cystic fibrosis

65
Q

Xray pattern in Pseudomonas
X ray pattern in Staph

Both are
Necrotizing pneumonia

A

Fleur de lis pattern

Pneumatocoele by Panton Valentin Leukocidin

66
Q

Pathognomonic of neuroblastoma*

A

Homer-Wright pseudorosettes

67
Q

Tumor cells are concentrically arranged about a central space*

A

Homer-Wright Pseudorosettes

68
Q

Port-Wine stain

Brain tumors

A

Sturge Webber Syndrome

69
Q

Disseminated neuroblastomas with multiple cutaneous metastases

A

Blueberry muffin baby

70
Q

Most common teratoma of adult

A

Ovarian teratoma

71
Q

Tumors with Homer-Wright Pseudorosettes

A

Neuroblastoma
Medulloblastoma
Primitive Neuroectodermal Tumors (PNETs)

72
Q

Retinoblastoma histopathologic landmark*

A

Flexner-Wintersteiner Rosette

73
Q

Ependymoma histologic landmark

A

True Ependymal Rosette

74
Q

Ovarian teratoma
Headache, Catatonia
Behavioral changes
Seizure

A

Anti-NMDA Encephalitis

75
Q 
Also seen in ependymomas
medulloblastoma
PNET
Central neurocytomas
Glioblastomas
A

Perivascular pseudorosette

76
Q

Most common primary renal tumor of childhood
Peak incidence: 2.5 years
Mutations in WT1 gene
Involve both kidneys: simultaneously (synchronous), one after the other (metachronous)?

A

Wilms tumor

Nephroblastoma

77
Q

Most common adrenal medulla tumor in adults

A

Pheochromocytoma

78
Q

C-MYC Gene is associated

C-mice kitkit

A

Burkitt lymphomas

Starry-Sky Appearance

79
Q 
Paraneoplastic syndrome
Neuroblastoma
Ataxia
Myoclonus 
Dancing eyes, dancing feet syndrome
A

Opsoclonus-myoclonus-Ataxia (OMAS) Syndrome
Kinsbourne syndrome
Dancing Eyes

80
Q

Specialized epithelium you will encounter in the ventricles of the brain and central canal of spinal cord

A

Ependyma

81
Q

Triphasic nephroblastoma comprises three elements

A

Blastema (least differentiated component, most malignant)
Mesenchyme (stroma)
Epithelium

BEM

82
Q

Most common site of metastases of nephroblastoma

A

Lungs

83
Q

Most common sites of metastasis in neuroblastoma

A

Bone

Bone marrow

Pathology (24 decks)

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