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Pathology > Diseases of Infancy and Childhood > Flashcards

Diseases of Infancy and Childhood Flashcards

1
Q

What is a common cause of Oligohydramnios (Potter) sequence?

A

Decreased fetal renal blood flow leading to decreased urine production

(Less fluid volume than expected for fetal age)

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2
Q

What is the cause of Polyhydramnios Sequence?

A
  • Decreased fetal swallowing
  • Increased fetal urination
  • (amniotic fluid volume greater than expected
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3
Q

What is the treatment for polyhydramnios?

A
  • Physical reduction
  • Prostaglandin synthetase inhibitors
    • indomethacin
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4
Q

What are the long-term effects of congenital rubella syndrome?

A
  • Hearing loss
  • Cataract formation
  • Retardation (growth and mental)
  • Congenital heart disease
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5
Q

Infant presents at birth with jaundice, hepatosplenomegaly, and anemia. What is the disease?

A

Cytomegalic inclusion disease

CMV

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6
Q

Two month old infant presents with notched central incisors, interstitial keratitis with blindness, and deafness. What is the disease? What is this triad called? What is the cause?

A

Late congenital syphilis

Hutchinson triad

Treponema pallidum

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7
Q

What can be caused by radiation exposure during organogenesis?

A

Blindness

Spina bifida

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8
Q

What are some long term effects of maternal hyperglycemia- induced hyperinsulinemia on the infant?

A

Cardiac anomalies

Neural tube defects

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9
Q

When is a fetus most susceptible to teratogens?

A

3rd-9th weeks

(organogenesis)

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10
Q

What virus causes fetal hydrops?

A

Parvovirus B19

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11
Q

What are the defects resulting from retinoic acid embryopathy? What is the cause?

A
  • Defects:
    • CNS
    • Cardiac
    • Craniofacial
  • Cause:
    • Retinoid acid changes HOX gene expression
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12
Q

What is the function of HOX genes?

A

Patterning of limbs, vertebrae, and craniofacial structures

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13
Q

What is considered a preterm birth?

A

before 37 weeks

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14
Q

What is considered a post-term birth?

A

after 42 weeks

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15
Q

Organisms associated with intrauterine infection are:

A
  1. Ureaplasma
  2. Mycoplasma
  3. Trichomonas
  4. Neisseria gonorrhoeae
  5. Chlamydia trachomatis

Induce labor by:

  • Release collagenases/ elastases
    • membrane rupture
  • Release prostaglandins
    • stimulate contractions
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16
Q

What is the cause of proportionate fetal growth restriction (FGR)? Of disproportionate FGR?

A
  • Proportionate:
    • fetal factors
  • Disproportionate
    • Placental factors
    • Multiple gestation
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17
Q

What is Caput succedaneum?

A

accumulation of interstitial fluid in the soft tissues of the scalp

(where head began to enter uterine canal)

18
Q

What is a cephalhematoma?

A

Subperiosteal collection of blood

(birth injury)

19
Q

How is lung maturity assessed in the fetus?

A

analyzing amniotic fluid phospholipids

20
Q

What is used to induce surfactant production in the newborn?

A

corticosterioids

21
Q

What are the complications of prolonged oxygen therapy on infants (oxygen toxicity)?

A
  • Retrolental fibroplasia (retinopathy)
    • neovascularization (increased VEGF) of retina
    • detachment and blindness may result
  • Bronchopulmonary dysplasia
    • decrease in the number of alveoli
22
Q

Newborn presents with bloody diarrhea and abdominal distention. Abdominal radiographs show gas within the intestinal wall. What is the disease? With what is it associated?

A
  • Disease: Necrotizing enterocolitis
  • Low birthweight premature infants
23
Q

What is hydrops fetalis?

A

Generalized accumulation of edema fluid

Lethal

24
Q

What are the causes of nonimmune hydrops?

A
  • CV malformations
  • Chromosomal abnormality
    • Turner Syndrome
    • Trisomies 21 and 18
  • Fetal anemia
25
Q

Erythroblastosis Fetalis can cause what type of hydrops?

A

Immune hydrops

26
Q

Which virus can cause nonimmune hydrops? What is the path?

A

Parvovirus B19

  • Path:
    • replicates in erythroid precursors
      • arrests maturation
      • peripheral rim of residual chromatin around nucleus
    • aplastic anemia
27
Q

Infant presents with a strong musty odor, seizures, decreased pigmentation of hair and skin, and mental retardation. What is the disease?

A

Phenylketonuria

  • Phenylalanine hydroxylase deficiency
28
Q

Infant presents with hepatomegaly, cataracts, CNS manifestations, and aminoaciduria. What is the disease? What is the cause?

A

Galactosemia

  • Cause: deficiency in gal-1-P uridyl transferase or galactokinase
29
Q

What is meconium ileus? With what condition is it associated?

A
  • Obstruction of the small intestine from thick plugs of mucus
  • Associated with cystic fibrosis in infants
30
Q

What causes male infertility in CF patients?

A

Bilateral loss of the vas deferens

31
Q

What is the pahogenesis of SIDS?

A

medullary 5-HT dysfunction

Hypoplasia of the arcuate nucleus

32
Q

What are choristomas?

A

aggregations of normal cells in an abnormal location

usually no clinical significance

33
Q

What are hamartomas?

A

Focal overgrowth of cells and tissues native to the organ in which it occurs

benign

34
Q

What are teratomas? What is the most common type?

A

Germ cell tumor

Most common: sacococcygeal

35
Q

Elevated blood levels of catecholamines signals which type of cancer?

A

Neuroblastoma

(normally occurs in adrenal glands)

36
Q

Where does neuroblastoma normally arise?

A

Adrenal gland

37
Q

On biopsy of a neoplasm, small blue cells arranged in rosettes (Homer-Wright pseudorosettes) are seen. What is the neoplams?

A

Neuroblastoma

38
Q

What is the outcome of neuroblastoma patients associated with the following:

  1. Hyper-diploidy
  2. Near-triploidy
  3. Diploidy or near-diploidy
  4. near-tetraploidy
  5. N-myc gene amplification
A
  • Hyper-diploidy (incomplete set)
    • good
  • Near-triploidy
    • good
  • Diploidy or near-diploidy
    • bad
  • near-tetraploidy
    • bad
  • N-myc gene amplification
    • worse with more copies present
39
Q

Patient presents with poor vision, strabismus, and a whitish hue to the pupil. Biopsy of the neoplasm shows clusters of cells arranged around a central lumen (Flexner-Wintersteiner rosettes). What is the disease?

A

Retinoblastoma

40
Q

Patient presents with large abdominal mass, hematuria, abdominal pain, and intestinal obstruction. What type of neoplasm could be the cause?

A

Wilm’s tumor

  • Tumor of the kidney
  • Precursor lesions: nephrogenic rests
  • Histology:
    • combination of blastemal, stromal, and epithelial cell types
41
Q

Patient presents with a painful, enlarging mass of the leg. The area is warm and swollen. This could be the result of which type of cancer? What is the genetic cause?

A

Ewing’s sarcoma

  • Cause: t (11; 22) of FL-1 near EWS gene
  • Bone cancer

Pathology (27 decks)

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