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Pediatrics Flashcards

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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
Most common finding in SIDS
Multiple petechiae Others: vascular engorgement pulmonary edema astrogliosis of brain stem and cerebellum
26
Hypoplasia of arcuate nucleus Persistence of hepatic extramedullary hematopoiesis Persistence of periadrenal brown fat
Sudden Infant Death Syndrome
27
What is the only safe sleeping position for infants that reduces the incidence of sudden infant death syndrome?
Supine position
28
Complications of oxygen therapy
Retrolental fibroplasia (retinopathy of prematurity) VEGF causes retinal neurovascularization Bronchopulmonary dysplasia
29
VEGF is also called
Vascular permeability factor
30
Decrease in alveolar septation Fibrosis Dysmorphic capillary configuration due to immature antioxidant defenses
Bronchopulmonary dysplasia
31
Breakdown of mucosal barrier functions permits transluminal migration of bacteria Most common surgical emergency in neonate
Necrotizing enterocolitis
32
Most important risk factor for N.E.C.
Prematurity
33
Bloody stool Abdominal distention Development of circulatory collapse
Necrotizing enterocolitis
34
Gas within intestinal walls
pneumatosis intestinalis
35
by hemolytic disease caused by blood group incompatibility
Immune hydrops
36
May also be called by Parvovirus B19 infection or Twin-twin transfusion syndrome
Non-immune hydrops
37
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
Hydrops fetalis
38
Immune hydrops prevention
Administration of RhIg to Rh negative mothers at 28 weeks AOG, within 72h of delivery or following abortions
39
Compensatory hyperplasia of bone marrow results in
Hydrops fetalis Erythroblastosis fetalis large numbers of rbcs
40
``` Kernicterus Hemolytic anemia Jaundice Chorea Athetosis ```
Erythroblastosis fetalis
41
Kericterus has special predilection to
``` basal ganglia thalamus cerebellum cerebral gray matter SC ```
42
Bilirubin deposits in basal ganglia of kernicterus baby is
>20 mg/dl
43
Autosomal recessive Mutations of gene encoding phenylalanine hydroxylase Inability to convert phenylalanine into tyrosine Excess phenylalanine metabolites cause brain damage and mental retardation
Phenylketonuria
44
Imparts a strong musty or mousy odor to affected infants
Phenylacetic acid
45
Galactose-1-phosphate uridyl transferase | Galactokinase
Galactosemia
46
Precursor of tyrosine Rate limiting enzyme? Cofactor?
Phenylalanine Phenylalanine hydroxylase Tetrahydrobiopterine
47
Most common galactosemia variant
Galactose 1-phosphate uridyl transferase
48
``` E coli septicemia Hepatomegaly Cataract Failure to thrive Mental retardation ```
Galactosemia
49
Tx for PKU
Sapropterin Dihydrochloride (Tetrahydrobiopterin BH4 THB) to decrease phenylalanine
50
Disorder of ion transport in epithelial cells Autosomal recessive transmission Most common lethal genetic disease that affects Caucasian populations
Cystic fibrosis
51
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
Cystic fibrosis
52
Atrophy of exocrine pancreas Meconium ileus (thick viscid mucus plugs causing SBO in infants) Respiratory infections Infertility (azoospermia, bilateral absence of vas deferens)
Cystic fibrosis
53
Cataract is galactosemia is caused by accumulation of In DM?
Galactitol in lens Oil drop cataract Sorbitol
54
Respiratory infections in cystic fibrosis are most commonly due to
Pseudomonas aeruginosa
55
Gene erroneous in cystic fibrosis
CFTR Gene | Cystic fibrosis transmembrane regulator gene
56
Most common lethal genetic disease in Caucasians
Cystic fibrosis
57
Most common vascular tumors of infancy | Flat larger lesions (port-wine stain)
Hemangiomas
58
Most common teratomas of childhood | Germ cell tumor
Sacrococcygeal teratoma Composed of developed tissues/organs Hair Teeth Muscle Bone
59
Forms of teratoma
Mature Immature Unequivocally malignant
60
Tumors of sympathetic ganglia and adrenal medulla Derived from primodial neural crest cells Amplification of N-MYC oncogene Peak age at 18 months
Neuroblastoma
61
Most common extracranial solid tumor of childhood
Neuroblastoma
62
Gene amplification of | Neuroblastoma
N-MYC oncogene
63
Multipel fluid loops of intestine | Meconium ileus on XRAY
Soap bubble sign
64
Meconeum ileus is associated to
Cystic fibrosis
65
Xray pattern in Pseudomonas X ray pattern in Staph Both are Necrotizing pneumonia
Fleur de lis pattern Pneumatocoele by Panton Valentin Leukocidin
66
Pathognomonic of neuroblastoma*
Homer-Wright pseudorosettes
67
Tumor cells are concentrically arranged about a central space*
Homer-Wright Pseudorosettes
68
Port-Wine stain | Brain tumors
Sturge Webber Syndrome
69
Disseminated neuroblastomas with multiple cutaneous metastases
Blueberry muffin baby
70
Most common teratoma of adult
Ovarian teratoma
71
Tumors with Homer-Wright Pseudorosettes
Neuroblastoma Medulloblastoma Primitive Neuroectodermal Tumors (PNETs)
72
Retinoblastoma histopathologic landmark*
Flexner-Wintersteiner Rosette
73
Ependymoma histologic landmark
True Ependymal Rosette
74
Ovarian teratoma Headache, Catatonia Behavioral changes Seizure
Anti-NMDA Encephalitis
75
``` Also seen in ependymomas medulloblastoma PNET Central neurocytomas Glioblastomas ```
Perivascular pseudorosette
76
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)?
Wilms tumor | Nephroblastoma
77
Most common adrenal medulla tumor in adults
Pheochromocytoma
78
C-MYC Gene is associated C-mice kitkit
Burkitt lymphomas | Starry-Sky Appearance
79
``` Paraneoplastic syndrome Neuroblastoma Ataxia Myoclonus Dancing eyes, dancing feet syndrome ```
Opsoclonus-myoclonus-Ataxia (OMAS) Syndrome Kinsbourne syndrome Dancing Eyes
80
Specialized epithelium you will encounter in the ventricles of the brain and central canal of spinal cord
Ependyma
81
Triphasic nephroblastoma comprises three elements
Blastema (least differentiated component, most malignant) Mesenchyme (stroma) Epithelium BEM
82
Most common site of metastases of nephroblastoma
Lungs
83
Most common sites of metastasis in neuroblastoma
Bone | Bone marrow

Pathology (24 decks)

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