Chapter 10 - Diseases of Infancy and Childhoood Flashcards

1
Q

Which of the following are TRUE about errors in morphogenesis?

A. Malformations represent secondary errors of morphogenesis in w/c there is an intrinsically abnormal developmental process
B. Disruptions result from primary destruction of an organ or body region that was previous normal in development
C. Deformations represent an intrinsic disturbance of development rather than an extrinsic error of morphogenesis
D. A sequence is a cascade of anomalies triggered by one or more initiating aberration
E. Two of the options
F. None of the options
G. All of the options

A

F. None of the options

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2
Q

Oligohydramnios is an example of which of the following errors in morphogenesis?

A. Malformation
B. Disruption
C. Deformation
D. Sequence
E. Two of the options
F. None of the options
A

D. Sequence

Malformation = congenital heart defects and anencephaly
Disruption = amniotic bands
Deformation = club feet
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3
Q

Potter sequence is also called which of the following?

A. Hydramnios
B. Polyhydramnios
C. Oligohydramnios
D. Anhydramnios

A

C. Oligohydramnios

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4
Q

All of the following results from fetal compression EXCEPT:

A. Altered facies
B. Positioning defects of feet and hands
C. Breech presentation
D. Pulmonary hypoplasia
E. Two of the options
F. None of the options
A

F. None of the options

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5
Q

Which of the following is the most common cause of congenital anomalies in live-born infants?

A. Genetic
B. Environmental
C. Multifactorial
D. Unknown

A

D. Unknown

Multifactorial = most common genetic etiology

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6
Q

Which of the following is the most common developmental defect of the forebrain and midface in humans?

A. Anencephaly
B. Holoprosencephaly
C. Schizencephaly
D. Lissencephaly

A

B. Holoprosencephaly

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7
Q

The embryo is extremely susceptible to teratogenesis in between which of the following weeks?

A. 2nd and 8th
B. 3rd and 9th
C. 1st and 6th
D. 1st and 8th
E. 3rd and 7th
A

B. 3rd and 9th

Peak sensitivity = b/w 4th and 5th weeks

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8
Q

Holoprosencephaly may be caused by which of the following teratogens?

A. Valproic acid
B. Cyclopamine
C. Retinol
D. Retinoic acid
E. All of the options
A

B. Cyclopamine

Cyclopamine = holoprosencephaly and cyclopia
Valproic acid = patterning of limbs disruption
Vitamin A (retinol) = eyes, genitourinary, CVS, diaphragm, lungs
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9
Q

HOX genes are responsible in which of the following processes?

A. Growth and maturation of the organs
B. Patterning of limbs, vertebrae, and craniofacial structures
C. Palatogenesis
D. Masculinization
E. All of the options
A

B. Patterning of limbs, vertebrae, and craniofacial structures

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10
Q

Prematurity is defined by a gestational age of which of the following?

A. Less than 36 weeks
B. Less than 37 weeks
C. Less than 38 weeks
D. Less than 39 weeks
E. Less than 40 weeks
A

B. Less than 37 weeks

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11
Q

Which of the following is the dominant organism found in “bacterial vaginosis”, that is responsible for intrauterine infections?

A. Ureaplasma urealyticum
B. Mycoplasma hominis
C. Gardnerella vaginalis
D. Trichomonas vaginalis
E. Chlamydia trachomatis
A

C. Gardnerella vaginalis

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12
Q

Hazards of prematurity may give rise to all of the following EXCEPT:

A. Neonatal respiratory distress syndrome
B. Hyaline membrane disease
C. Necrotizing enterocolitis (NEC)
D. Sepsis and hemorrhage
E. Two of the options
F. None of the options
A

F. None of the options

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13
Q

All of the following are most commonly responsible for FGR (fetal growth restriction) EXCEPT:

A. Toxoplasmosis
B. Syphilis
C. Rubella
D. Cytomegalovirus
E. Herpes virus
F. Two of the options
G. None of the options
A

G. None of the options

TORCH group of infections

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14
Q

Which of the following surfactant proteins is/are involved in reduction of surface tension in the lung alveoli?

A. SP-A
B. SP-B
C. SP-C
D. SP-D
E. Two of the options
F. All of the options
A

E. Two of the options

SP-A & SP-D = innate immunity
SP-B & SP-C = reduction of surface tension

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15
Q

All of the following are clinical features associated with NRDS EXCEPT:

A. Preterm
B. Male gender
C. Maternal diabetes
D. Caesarean delivery
E. Two of the options
F. None of the options
A

F. None of the options

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16
Q

All of the following are TRUE about necrotizing enterocolitis EXCEPT:

A. Most common in premature infants
B. Directly proportional to the gestational age
C. Occurs in infants <1500g
D. Most cases are associated with enteral feeding
E. Two of the options
F. None of the options

A

B. Directly proportional to the gestational age

17
Q

Which of the following shows pneumatosis intestinalis in abdominal radiographs?

A. Transcervical infection
B. Transplacental infection
C. Hyaline membrane disease
D. Necrotizing enterocolitis
E. Fetal hydrops
A

D. Necrotizing enterocolitis

18
Q

All of the following are TRUE about clinical features of NEC EXCEPT:

A. Abdominal distention
B. Bloody stools
C. Circulatory collapse
D. Kidney injury
E. None of the options
A

D. Kidney injury

NEC = “ABC”

19
Q

All of the following are TRUE about transcervical infections EXCEPT:

A. Also called hematologic infections
B. Commonly caused by most bacterial infections
C. Preterm birth is common consequence
D. Pneumonia, sepsis, and meningitis are the most common sequelae
E. Two of the options
F. None of the options

A

A. Also called hematologic infections

Transplacental:

Hematologic = Hepa B & HIV
Mostly caused by parasitic infections
Parvovirus B19
TORCH group of infections

20
Q

All of the following are TRUE about perinatal sepsis EXCEPT:

A. Early onset = within first 7 days of life
B. Late onset = from 7 days to 3 months
C. Signs and symptoms involve pneumonia, sepsis, and meningitis that occurs within 4 or 5 days of life
D. Group B strep is the most common cause of early onset
E. Listeria and Candida commonly cause late onset sepsis
F. Two of the options
G. None of the options

A

G. None of the options

21
Q

This is a hemolytic disease caused by blood group antigen incompatibility between mother and fetus.

A. Immune hydrops
B. Nonimmune hydrops
C. Fetal hydrops
D. Hemolytic anemia
E. All of the options
A

A. Immune hydrops

22
Q

All of the following are TRUE about immune hydrops EXCEPT:

A. There is an immunization of the mother by blood group antigens on fetal red cells
B. There is free passage of antibodies from the mother through the placenta to the fetus
C. Concurrent ABO incompatibility protects the mother against Rh immunization
D. The antibody response depends on the dose of immunization antigen
E. None of the options

A

E. None of the options

23
Q

The use of RhIg containing anti-D antibodies against immune hydrops is administered at what age of gestation?

A. 27 weeks
B. 28 weeks
C. 36 weeks
D. 37 weeks

A

B. 28 weeks

24
Q

All of the following are major causes of nonimmune hydrops EXCEPT:

A. CV defects
B. Chromosomal anomalies
C. Fetal anemia
D. Leukocytosis
E. None of the options
A

D. Leukocytosis

Non-immune = “CFC”

Most common cause = fetal anemia = thalassemia = deletion of four all globin genes

25
Q

Which of the following is the most serious threat in fetal hydrops?

A. Thalassemia
B. Kernicterus
C. Erythroblastosis fetalis
D. Tetralogy of fallot

A

B. Kernicterus

26
Q

All of the following are TRUE about PKU EXCEPT:

A. Autosomal dominant
B. Deficiency of phenylalanine hydroxylase (PAH)
C. Inability to convert phenylalanine into tyrosine
D. Impairment of brain development
E. None of the options

A

A. Autosomal dominant

Most inborn errors of metabolism are autosomal recessive or X-linked traits

27
Q

All of the following are TRUE about Galactosemia EXCEPT:

A. Autosomal recessive
B. Accumulation of galactose-1-phosphate
C. More common variant is total lack of GALT
D. Less common variant is deficiency of galactokinase which is not associated with intellectual disability
E. None of the options

A

E. None of the options

lack of GALT = hepatomegaly, cataract, alterations in CNS, vomiting and diarrhea after milk ingestion

28
Q

All of the following are TRUE about cystic fibrosis EXCEPT:

A. Also called as Mucoviscidosis
B. There is a primary defect in transport of chloride and bicarbonate ions
C. CTFR gene is location on chromosome 7q31.2
D. Most common lethal genetic disease that affects Caucasian populations
E. None of the options

A

E. None of the options

29
Q

As per cystic fibrosis, which class represents defective protein synthesis or null mutations?

A. Class I
B. Class II
C. Class III
D. Class IV
E. Class V
A

A. Class I

I = protein synthesis/null mutations
II = protein folding/processing mutations
III = regulation/gating mutations
IV = conductance/conduction mutations
V = abundance/production mutations
VI = CFTR stability/instability mutations
30
Q

Which of the following are the most serious complications of cystic fibrosis?

A. Pancreatic abnormalities
B. Liver involvement
C. Pulmonary changes
D. Azoospermia and infertility
E. Two of the options
F. All of the options
A

C. Pulmonary changes

31
Q

All of the following are the most common organisms responsible for lung infections in cystic fibrosis EXCEPT:

A. Staphylococcus aureus
B. Haemophilus influenzae
C. Pseudomonas aeruginosa
D. Streptococcus neoformans

A

D. Streptococcus neoformans

32
Q

Sudden infant death syndrome occurs on infant at what age?

A. <6 months
B. <1 year
C. <1.5 years
D. <2 years

A

B. <1 year

SIDS = unexplained 
SUID = anatomic/biochemical basis
33
Q

This refers to microscopically normal cells present in abnormal locations.

A. Hamartoma
B. Choristoma
C. Adenoma
D. Teratoma
E. Hemangioma
A

B. Choristoma or heterotopia

Hamartoma = excessive, focal overgrowth of cells native to the organ

34
Q

Which of the following is the most common tumor of infancy?

A. Hamartoma
B. Choristoma
C. Adenoma
D. Teratoma
E. Hemangioma
A

E. Hemangioma

35
Q

Port-wine stains are found in what lesion?

A. Hamartoma
B. Choristoma
C. Adenoma
D. Teratoma
E. Hemangioma
A

E. Hemangioma