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PB+T > Ped Path > Flashcards

Ped Path Flashcards

0
Q

Disruption

A

Extrinsic, mechanical force altering the development

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1
Q

Deformation

A

Extrinsic, biomechanical force altering development

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2
Q

Malformation

A

Intrinsic/genetic force altering development

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3
Q

Malformation: epidemiology

A 
Idiopathic: 60%
Multifactorial: 25%
Chromosomal abnormalities: 10-15%
Maternal disease states: 6-8%
TORCHES: 2-3%
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4
Q

Viral susceptibility: time frame

A

From just before conception - 16 weeks

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5
Q

Alcohol as a teratogen

A

Disrupts signaling of retinoic acid and sonic hedgehog

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6
Q

Thalidomide teratogenicity

A

Upregulates WNT suppressors

-

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7
Q

Nicotine effect on fetus

A

SGE, prone to SIDS

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8
Q

Maternal diabetes effects

A

Hyperglycemia -> fetal hyperinsulinemia -> acts as GF increasing both size and rates of organ abnormalities

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9
Q

Sensitive period

A

3-8 weeks

- neurulation occurs earliest and has greatest susceptibility

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10
Q

Cyclopamine

A

Screws with sonic hedghog, can result in sever NTDs

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11
Q

Valproic acid as a teratogen

A

Causes mutations in the HOX genes

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12
Q

Vitamin A (retinol)

A

It’s essential for organogenesis, but too much can result in retinoic acid embryopathy. May be associated with messed up TGF-B

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13
Q

Gestation time: risk factors for early parturition

A

Prematurity is the second leading cause of infant mortality.

  • premature rupture of membranes and intramniotic infection are common causes
  • structural abnormalities
  • multiple gestations
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14
Q

Risk factors for newborns

A

Weight trumps appropriateness for age

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15
Q

Fetal growth restrictions; fetal and maternal factors

A
  • maternal factors: hypertension, preeclampsia, hypercoagulable states
  • fetal (represented by symmetric abnomalities) and placental (represented by asymmetric abnormalities) factors also play a role.
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16
Q

Risks for preterm infants (5); 2 organ systems, 2 systemic risks

A 
Hyaline membrane disease
Necrotizing enterocolitis
Sepsis 
Intervenous hemorrhage 
Long term complications
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17
Q

Neonatal RDS: etiology, risk factors (3)

A

Etiology: underdeveloped lung tissue -> little/no surfactant ->contributing to hyaline membrane disease
- male, c-section, maternal diabetes risk factors

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18
Q

Surfactant A/B vs. C/D

A

A/B - immune function: innate defense
C/D - reduction in surface tension
- surfactant controlled by SFTPB/SFTPC

19
Q

Chemical mediators if surfactant

A

TGF-B and corticosteroids stimulate it

Insulin inhibits it

20
Q

Bronchopulmonary dysplasia

A

Rare in infants over 1200 g

  • high lvls of O2 contribute to development
  • inflammatory cytokines increase
  • if they survive past 4 days recovery can be expected at ~ 31 weeks.
21
Q

Transcervical vs. transpalacental infections

A

Transcervical: mostly bacteria (some viruses like HSVII). In fetus infections is typically respiratory (inhaled amniotic fluid) and sepsis
- transplacental: most parasites, TORCHES viruses, listeria, treponema pallium

22
Q

TORCH effects

A

Fever, encephalitis, hepatosplenomegaly, pneumonitis, myocarditis, hemolytic anemia, vesicular damage, and hemorrhagic skin lesions

23
Q

Immune hydrops

A
  • aka hemolytic disease of the new born. Can result in anemia, kernicterus, or hydrops from cardiac decompensation
  • The D antigen is the major cause of Rh incompatibility
  • occurs only after significant trans-placental bleeding
  • administration of Anti Ab Ig happens at 28 weeks to prevent sensitization, and again within 72 hours of delivery.
24
Q

PKU

A
  • Most commonly it the Absence of phenylalanine hydroxylase-> accumulation of Phe
  • it may also be reduced TH4 (phe doesn’t get oxidized to tyrosine) this form will not be treated by reducing phe intake.
  • control of maternal PKU is ESSENTIAL during pregnancy. Elevated Phe lvls are devastating to baby.
25
Q

Galactosemia

A
  • primarily a deficiency of GALT
  • leads to the G-1-P accumulates and is toxic. (Cataracts and DD are two giveaways)
  • hepatomegaly happens early, FTT is also early
  • may also have frequent E. coli septicemia
  • trt by removing galactose from diet
26
Q

Cystic fibrosis

A
  • CFTR gene mutation -> bad chloride channels as well as ENaCs, and k channels
  • channels are cAMP mediated
  • the sweat gland ENaC channels aren’t CFTR controlled, resulting in high sodium AND high chloride sweat
  • decreased luminal pH from lack of Bicarbonate transport contributes to obstructions
27
Q

6 classes of CF

A

I. Defective protein synthesis
II. Abnormal protein folding: most common (delta F508)
III. Defective regulation
IV. Decreased conductance
V. Reduced abundance
VI. Altered regulation of separate ion channels
- types I,II and III are the most

28
Q

Phenotypic differences among CF PTs.

A

TGF- B and MBL are both influenced by the CFTR gene and are involved in innate immunity.

29
Q

Heterotropia

A

Heterotrophic tissue is normal tissue in a an abnormal place

  • lipoma
  • hamartoma
30
Q

Anaplastic lymphoma kinase (ALK-1)

A

The major cause of neuroblastomas

31
Q

Homer-wright pseudorossets

A

Small, scant cytoplasm containing cells compacted in a tumor represent a histologically classical neuroblastoma

32
Q

Ganglioma

A

Similar to Neuroblastoma accept the ganglioma contains mature ganglion cells

33
Q

Schwannian stroma

A

Represents a more favorable prognosis

34
Q

Neuroblastoma prognosis

A

Age and stage are most important prognostic factors
- 18 mo is the turning point
Amplification of the N-MYC gene is the most important independent factor

35
Q

N-MYC in neuroblastomas

A

Hemizigous deletion of distal short arm found in 25-35% of primary tumors
Partial gain of distal long arm is present in up to 50% of tumors

36
Q

Wilms tumor

A
  • renal tumor that typically occurs from Age 2-5 up to about age 9
  • follows two hit hypothesis
  • WAGR syndrome has higher risk of developing the Wilms tumor
37
Q

Denys-drash syndrome

A
  • male pseudohermaphroditism
  • early onset neuropathy
  • Germ line abnormalities in WT1 gene (biallelic activation)
38
Q

WT-1

A

Gene associated with development of Wilms tumor

39
Q

Beckwith-weiderman syndrome

A
  • organomegaly
  • microglossia
  • hemihypertrophy
  • gene abnormality near locus of WT-1: Associated with Wilms tumor
40
Q

IGF-2

A

Paternally imprinted insulin like growth factor

- when its over expressed can lead to development of Wilms tumor

41
Q

Nephrogenic rests

A

Putative precursor lesions to a Wilms tumor

- Associated with some unilateral Wilms tumors and almost all bilateral Wilms tumors

42
Q

Oxygen toxicity

A

Contribute to Bronchopulmonary dysplasia

43
Q

Frequent early E. coli septicemia

A

May indicate Galactosemia, Especially if accompanied by failure to thrive

44
Q

Male, c-section, maternal diabetes

A

Risk factor for neonatal RDS

45
Q

Symmetric vs. asymmetric fetal abnormalities

A

Symmetric: fetal factor
Asymmetric: placental factor

46
Q

Maternal fetal growth restrictions

A

It’s all about blood flow: HTN, hypercoagulable states

PB+T (20 decks)

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