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Written MFM Boards > Written MFM Boards 2 > Flashcards

Written MFM Boards 2 Flashcards

1
Q

Method to assess effect of different factors on survival

A

Cox regression

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2
Q

Reported neonatal toxicity with SSRI use

A

persistent pulmonary HTN (right-left shunting across ovale and PDA can lead to fetal hypoxia)

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3
Q

Rate limiting step in T3/T4 production

A

Trapping of Iodide in thyroid gland

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4
Q

What is function of TPO enzyme

A

Converts Iodide to Iodine in the thyroid gland

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5
Q

What is function of thyroglobulin

A

Stores T3 and T4 in thyroid gland

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6
Q

Thyroglobulin vs thyroid binding globulin

A

Thyroglobulin - stores T3/T4 in thyroid gland

TBG - protein that T3/T4 are bound to in serum

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7
Q

Type of receptor T3 and T4 bind to

A

Nuclear receptor

T3 affinity >T4

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8
Q

Most accurate thyroid testing in critically ill patients?

A

Free T4

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9
Q

What thyroid level is typically elevated in critical illness, as a result of physiology not pathology?

A

rT3

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10
Q 
Thyroid hormone changes in pregnancy:
TRH
TSH
Free T3/T4
Total T3/T4
Thyroid binding globulin
A

TRH/TSH - decreased
Free T3/T4 - same or marginally increased
Total T3/T4 - increase
TBG - increase

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11
Q

Enzymes (deiodinases) involved in peripheral conversion of T4–>T3

A

Type I - unchanged in pregnancy
Type II - in placenta, maintains local placental levels of T3
Type III - in placenta, makes rT3

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12
Q

Thyroid hormones that cross the placenta

A

T4

TRH

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13
Q

Thyroid hormone that does not cross placenta

A

TSH

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14
Q

Amniotic fluid levels of thyroid hormone are reflective of maternal or fetal thyroid serum levels

A

Fetal

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15
Q

What happens to neonatal thyroid levels following delivery and why?

A

transient hyperthyroxinemia – thought to help with thermoregulation

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16
Q

Anti-TPO but no overt hypothyroidism, at risk of what

A

Postpartum thyroiditis

Hypothyroidism

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17
Q

Iodine intake recommendation pregnancy/lactation

A

WHO - 250ug daily
ATA - 150ug daily
IOM - 220ug pregnancy and 290ug lactation daily

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18
Q

When is fetal TSH present? Fetal T3/T4?

A

TSH at 10-12 weeks as fetal thyroid can capture iodide, but not much T3/T4 until 18-20 weeks

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19
Q

How to test for iodine deficiency

A

Urine iodine levels:
24hr secretion >100ug intake is sufficient
<50ug moderate deficiency

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20
Q

Iodine deficiency can be exacerbated by what deficiency

A

Selenium

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21
Q

Pregnancy complications associated with hypothyroidism

A 
Pregnancy loss
Stillbirth
LBW
Preeclampsia
Abruptions
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22
Q

When to test for thyroid stimulating antibodies in pregnancy

A

After 20 weeks, as high hCG can falsely low Ab

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23
Q

Hyperthyroidism treatment in pregnancy

A

1st tri: PTU, avoid long term use hepatotoxicity

2nd/3rd tri: MMI, avoid in 1st tri because of choanal atresia, aplasia cutis, TE fistula

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24
Q

PTU:MMI dose equivalents

A

20:1

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25
Q

Fetal hypothyroid signs

A

Goiter
Bradycardia
Growth restriction

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26
Q

How are Iodides used for hyperthyroidism treatment?

A
  • Prevent T3/T4 from being released from maternal thyroid
  • Use PTU/MMI before using Iodides
  • Don’t use for more than 2 weeks as crosses placenta and can lead to fetal goiter
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27
Q

Fetal hyperthyroidism symptoms

A 
Goiter
Tachycardia
Advanced bone age
Hydrops
Craniosynostosis
Growth restriction
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28
Q

Clinical manifestations of thyroid storm

A
  • Thermoregulatory dysfunction
  • CNS effects: agitation, delirium, coma
  • GI dysfunction
  • CV dysfunction: tachycardia, arrhythmia
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29
Q

Lab value predictive of high risk fetal hyperthyroidism

A

> 300% TSI

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30
Q

Protocol for Thyroid Storm treatment

A
  1. B-blocker
  2. PTU
  3. 1 hr after PTU given Potassium Iodide
  4. Dexamethasone
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31
Q

Congenital myotonic dystrophy inheritance pattern

A

AD - trinucleotide repeat

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32
Q

Ultrasound findings of congenital myotonic dystrophy

A 
Polyhydramnios
DFM
PTB 
FGR
Club foot
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33
Q

Pathophysiology of TTP

A

Platelet aggregates form leading to micro-occlusion of vessels

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34
Q

Vessels predominately effected by TTP

A

Brain and kidney

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35
Q

Classic pentad of clinical findings in TTP

A
  1. Fever
  2. Kidney damage
  3. Neuro dysfunction
  4. Hemolytic anemia
  5. Thrombocytopenia

**Anemia, thrombocytopenia and neuro changes are most common

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36
Q

TTP caused by antibodies to

A

ADAMTS-13

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37
Q

Serum finding that can aid in differentiating HELLP and TTP

A

antithrombin III level decreased in HELLP

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38
Q

Treatment for TTP

A

Plasmapheresis and plasma exchange with platelet-poor FFP
Typically followed by steroids

Treament 5d if rapid complete response, 3-4weeks if partial response

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39
Q

Treatment for refractory TTP

A

Vincristine
Azathioprine
Splenectomy

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40
Q

Lab values to help differentiate between HUS/TTP and AFLP

A

AFLP - decrease antithrombin III and coagulopathy

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41
Q

Most specific maternal serum testing for acute CMV infection

A

Low avidity IgG (2-4months)

IgM positive - can be positive for long time, can be false positive from other viruses, can be positive from reactivation

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42
Q

Bacterial enzyme linked to preterm labor

A

Phospholipase A2

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43
Q

List fetal organs that receive most cardiac output

A
  1. placenta
  2. lower body
  3. upper body
  4. lungs
  5. GI tract
  6. Heart/brain/kidneys
  7. Liver
  8. Spleen
  9. Adrenas
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44
Q

Part of fetal heart with highest PO2

A

Left atrium

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45
Q

Po2 values of umbilical artery and vein on cord gases

A 
vein = 28-32
artery = 19
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46
Q

Warfarin embryopathy risk is highest at what GA

A

6-12 weeks

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47
Q

Risk of congenital anomaly when Hgb A1c < or > 8.5%

A

<8.5% –> 3% risk

>8.5% –> 22% risk

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48
Q

Causes of low estriol (2)

A
  1. X-linked ichthyosis: due to placental sulfatase deficiency
  2. SLOS: due to mutation in 3-betaOH-7-dehydrocholesterol reductase
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49
Q

Clinical findings of SLOS

A 
Polydactyly/Syndactyly
Microcephaly
FGR
Cleft lip/palate
Ambiguous genitalia
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50
Q

Level of what protein is elevated in SLOS? What is low?

A

7-DHC levels in amniotic fluid

Cholesterol levels are low

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51
Q

Aneuploidies associated with low PAPP-A

A

T21 and T18 (very low)

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52
Q

OB complications associated with low PAPP-A

A 
pre-eclampsia
FGR
PTB
SAB 
IUFD
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53
Q

AFP made by what structures

A

Yolk sac
Fetal GI tract
Fetal liver

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54
Q

Causes of elevated MSAFP

A
  1. Fetal anomaly - ONTD, abdominal wall defect
  2. Congenital nephrosis
  3. Teratomas
  4. Incorrect dating
  5. Multifetal gestation
  6. Fetal death
  7. PAS
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55
Q

How do the following affect MSAFP levels

  1. weight
  2. DM
  3. Race
  4. GA
A
  1. larger weight means lower MSAFP (more diluted)
  2. DM have lower MSAFP
  3. Black women have higher MSAFP
  4. Increase in MSAFP with increasing GA
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56
Q

OB complications associated with elevated MSAFP

A 
FGR
Preeclampsia
PTB
IUFD
Abruption
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57
Q

Serum screening results in T21

A

PAPP-A, Estriol, AFP - low

bHCG, Inhibin - high

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58
Q

CRL limits for NT measurement

A

45-84mm

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59
Q

1st trimester serum markers

A

PAPP-A and b-hCG

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60
Q

2nd trimester serum markers

A

b-hCG
Inhibin A
Estriol
AFP

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61
Q

Soft markers with highest to lowest LR for aneploidy

A
  1. Nuchal fold
  2. Absent nasal bone
  3. Echogenic bowel
  4. Short humerus
  5. Short femur
  6. Echogenic intracardiac focus
  7. UTD
  8. CPC
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62
Q

Causes of low estriol on serum screening

A

T21
T18
Sulfatase deficiency
SLOS

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63
Q

To make it a quad screen which serum analyte is added

A

Inhibin A

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64
Q

Describe integrated screening

A

1st tri (NT+PAPP-A) and 2nd tri (quad screen) put together to give final risk, reported after completion of all tests

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65
Q

Describe stepwise versus contingent sequential screening

A

Stepwise – 1st tri risk reported if high, if not elevated then only risk after 2nd is reported

Contingent - 1st tri risk done if low then no further testing, if moderate then 2nd tri testing, if high then diagnostic testing recommended

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66
Q

What MoM of PAPP-A levels have highest association with placental insufficency

A

at or below 0.2

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67
Q

OB complications associated with high Inhibin A

A

Preeclampsia
FGR
IUFD
PTB

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68
Q

What does high hCG and high MSAFP indicate

A

Associated with OB complications

Confined placental mosaicism for T16

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69
Q

Carrier frequency for CF, SMA (white)

A

CF - 1:25

SMA - 1:50

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70
Q

Gene that causes SMA

A

SMN1

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71
Q

What is the difference between SMN1 copies in non-carriers and carriers

A

Noncarrier - two copies of SMN1 gene in trans (one on each chromosome)

Carrier - two copies of SMN1 gene in cis (both copies on one chromosome) OR one copy of SMN1 gene

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72
Q

What race has higher cis-carrier rate of SMN1

A

Blacks

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73
Q

Osteogenesis imperfecta is caused by what type of genetic mutation

A

AD mutation in Col1A

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74
Q

Achondroplasia is caused by what type of genetic defect

A

AD condition, mutation in FGFR3

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75
Q 
Differences between pre-renal and intrinsic AKI
BUN:Cr
Urine Na
FENa
Osmolality
Specific gravity
A 
Pre-renal:
BUN:Cr >20:1
Na <20
FENa <1%
Osm >500
SG >1.02
Intrinsic:
BUN:Cr 10:1
Na >40
FENa >2%
Osm <350
SG 1.01
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76
Q

What structures produce adipokines

A

Adipose tissue

Placenta

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77
Q

Leptin/Adiponectin — which is proinflammatory?

A

Leptin

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78
Q

In obesity what happens to Leptin/Adiponectin

A

Leptin – increases

Adiponectin – decreases

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79
Q

Elevated Leptin levels in pregnancy are associated with what OB complications

A

GDM

pre-eclampsia

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80
Q

Hormones regulating Calcium homeostasis

A

PTH – stimulates bone resorption

Vitamin D – stimulates GI absorption and bone resorb

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81
Q

Vit D IU recommendation in pregnancy/lactation

A

600IU

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82
Q

What hormone produced by breasts help increase Ca levels for lactation

A

PTHrp

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83
Q

T/F: placenta makes PTHrp

A

True

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84
Q

Clinical sx of hyperparathyroidism

A 
Stones
Weakness
Peptic ulcers
Pancreatitis
Constipation
Anorexia
Nausea/vomiting
HTN
Depression/Psychosis
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85
Q

Diff dx of hypercalcemia

A 
Hyperparathyroidism - secreting nodule
Malignancy
Granulomatous disease
Thyrotoxicosis 
Hypervitaminosis D or A
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86
Q

Neonatal complications of maternal hyperparathyroidism

A

Neonatal hypocalcemia and tetany

SAB and stillbirth

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87
Q

Medical management of hyperparathyroidism

A 
Hydration
Lasix
Phosphates
Calcitonin 
Bisphosphonates (not used in pregnancy)
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88
Q

Clinical sx of hypoparathyroidism

A

Tetany
Paresthesia
Mental changes
QT prolongation

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89
Q

Neonatal complications of maternal hypoparathyroidism

A

Bone demineralization
FGR
Neonatal hyperparathyroidism

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90
Q

Type of cells that make up 40% of the decidua

A

NK cells

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91
Q

Cells of pregnancy that do NOT have MHC class I molecules (HLA)

A

Syncytiotrophoblast

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92
Q

OB complications in IBD

A

SGA
PTB
Cesarean
Stillbirth

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93
Q

T/F: IBD tends to stay the same in terms of activity during pregnancy as it was pre-pregnancy

A

True

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94
Q

Possible side effects of prolonged steroid use

A

PPROM
GDM
Cleft palate

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95
Q

Women on Sulfasalazine should be advised to take what preconception and continue throughout pregnancy

A

Folic acid 2mg (x1 month preconception)

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96
Q

Risk of child having IBD if one or two parents have IBD

A

1 - 5%

2 - 30%

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97
Q

If someone presents with symptoms of IBD flare, important to rule out what as well

A

C. diff

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98
Q

T/F: Pregnancy can lead to decreased flares postpartum and longer term in IBD

A

True

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99
Q

Abxs of choice for endocarditis prophylaxis

A

Ampicillin
Ceftriaxone
Clindamycin

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100
Q

IV drug use is associated with endocarditis of which valve

A

Tricuspid valve

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101
Q

Major and minor criteria for infective endocarditis

A

Major – +blood cultures or vegetation in imaging

Minor – predisposing condition, fever, vascular phenomena, immunologic phenomena

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102
Q

Definite endocarditis is defined as what based on modified Duke

A
  • 2 major
  • 1 major and three minor
  • 5 minor
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103
Q

2 major functions of adiponectin

A
  1. insulin sensitization

2. anti-inflammatory properties

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104
Q

Conditions that low adiponectin levels are seen in

A

Obesity
DM
Metabolic syndrome

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105
Q

Most common (2) antigens causing alloimmunization

A
  1. Rh (D)

2. Kell

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106
Q

Sommatomamotropin levels are ____ in SGA and LGA pregnancies

A

Decreased in SGA

Increased in LGA

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107
Q

Are Ghrelin levels high or low in obese women

A

Low

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108
Q

Does Ghrelin stimulate or suppress appetite

A

stimulate appetite

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109
Q

What happens to Ghrelin levels over the course of pregnancy

A

Ghrelin levels high in 1st trimester and decreases over gestation

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110
Q

What happens to Leptin levels in pregnancy

A

Steadily increase

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111
Q

Conditions associated with high Leptin

A

Obesity
DM
GDM
Preeclampsia

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112
Q

OB complication associated with decreased adiponectin

A

LGA

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113
Q

Risk of Rh (D) alloimmunization after 1st pregnancy

A

~1%

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114
Q

Without Rhogam what % of Rh negative women will become sensitized

A

17%

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115
Q

Rhogam 300ug protects against how much fetal blood/RBC’s

A

30uL of whole blood or 15mL of RBCs

116
Q

Rh (D) critical titer

A

> 1:16

117
Q

Typical drop in Hct following fetal transfusions

A

1 point/day post-transfusion

118
Q

What is responsible for Na urinary excretion?

A

ANP

119
Q

What is responsible for urine Na retention

A

Aldosterone

120
Q

Congenital heart disease not usually associated with genetic syndrome

A

Heterotaxy

Transposition

121
Q

Congenital heart disease with highest rate of associated aneuploidy

A

AV canal defect

122
Q

Genetic disorders associated with fetal TOF

A

T21

DiGeorge

123
Q

Genetic syndrome associated with truncus arteriosus

A

22q11 deletion

124
Q

Folic acid supplementation recommendations:

(a) sickle cell
(b) twins
(c) singletons
(d) epilepsy

A

(a) 4-5mg/day
(b) 1mg
(c) 400ug
(d) 4mg

125
Q

How is diagnosis of active versus latent TB made?

A

Active - positive sputum culture

Latent - positive testing, no clinical sx, no evidence of active disease on CXR

126
Q

Latent TB treatment in pregnancy regimen

A

INH 300mg daily for 3-6 months

127
Q

Active TB in pregnancy treatment regimen

A

Same as non-pregnant

First 2 months: INH + rifampin + pyrazinamide + ethambutol
Next 4 months: INH + rifampin

128
Q

Medication that should be used as adjunct to INH

A

B6 – because INH can interfere with B6 metabolism

129
Q

INH side effect and how its monitored

A

Hepatitis – check LFTs and bilirubin prior to treatment and then monthly

130
Q

Why is active TB treatment 2-parts?

A

1st part = kill active organisms

2nd part = microbiologic cure

131
Q

TB drug treatment that is contraindicated in pregnancy

A

Streptomycin – associated w/ hearing loss

132
Q

pH of breast milk

A

7.0

133
Q

Location for listening to aortic, TC, mitral, pulmonary valve

A

Aortic - right upper sternal border
Pulmonary - left upper sternal border
TV - left sternal border, 4th ICS
Mitral - left midclavicular line, 5th ICS

134
Q

Most common valvular problem in chronic rheumatic heart disease

A

Mitral stenosis followed by aortic stenosis and aortic regurgitation

135
Q

Late valvular manifestation of chronic rheumatic disease

A

tricuspid regurgitation – secondary to right heart failure

136
Q

Inheritance of hypertrophic cardiomyopathy

A

AD, variable penetrance

137
Q

Anti-seizure meds that may worsen muscle weakness in Myasthenia Gravis

A

Phenytoin (Dilantin)

138
Q

Abx that can potentiate Magnesium neuroblockade

A

Gentamicin

139
Q

GA most asthma exacerbations occur

A

24-36 weeks

140
Q

Does FEV1 or PEF change in pregnancy

A

No

141
Q

Normal range of PEF

A

380-550L/min

142
Q

In acute asthma exacerbation, after giving a SABA what is considered a good response for at home management and what requires ER presentation

A

PEF is >80% predicted is good response, if PEF <50% then should present to ER

143
Q

Trisomy 18 findings

A 
FGR
Hypertonia
Micrognathia
Horseshoe kidney
Meckels diverticulum
Omphalocele
CDH
ONTD
Cardiac defects - VSD and PDA most common
Clenched hands, overlapping digits
Polyhydramnios
Rocker bottom feet
144
Q

Trisomy 13 findings

A 
Holoprosencephaly
Micro/anopthalmia
Cardiac defects -- HLHS
Enlarged echogenic kidneys
Postaxial polydactyly
FGR
145
Q

What % difference between pre and post-ductal neonatal O2 saturation is abnormal

A

10%

146
Q

What does a difference in pre and post ductal O2 saturation in neonates indicate

A

Right to left shunting

147
Q

What is TTN caused by

A

Continued lung fluid in the parenchyma

148
Q

What is genetic anticipation

A

phenotype get more severe and/or presents at a younger age with each generation

149
Q

How does cAMP lead to smooth muscle relaxation

A

cAMP is a second messenger that functions to reduce intracellular Calcium in smooth muscle cells

150
Q

Periventricular leukomalacia most common in what infection

A

Chorioamnionitis

151
Q

Advantages of delayed cord clamping in term neonate

A

Increased iron stores

Higher Hgb levels

152
Q

Disadvantages of delayed cord clamping

A

Increased risk of hyperbilirubinemia, polycythemia (SGA infants), reduced volume of umbilical blood available for harvesting

153
Q

Advantages of delayed cord clamping in preterm infants

A

Increased iron and Hgb levels with decreased need for transfusion
Reduce mortality
Improved transitional circulation
Decreased NEC and IVH

154
Q

Risk factors for spina bfida

A 
Folate deficiency
Pregestational DM
Obesity
Fever/hyperthermia
Epilepsy
155
Q

Autoimmune med associated with 25% teratogenicity risk

A

Mycophenolate – ear, eye lip/palate abnormalities

156
Q

True/False: Mitral/Aortic stenosis should be kept wet

A

True

157
Q

Infectious disease associated with placentomegaly and pale placenta

A

Syphilis

158
Q

Dermatoses associated with adverse fetal outcomes

A

Pustular psoriasis

Pemphigoid gestationalis

159
Q

Maternal congenital cardiac disease with highest risk of same cardiac disease in fetus

A
  1. Aortic Stenosis

2. AVSD

160
Q

Chance of fetal congenital heart disease with affected sibling

A

3%

161
Q

Chance of recurrent HLHS with affected sibling

A

8%

162
Q

Lupus flare associated with _____ levels of complement

A

low

163
Q

WBC count in lupus flare can be _____

A

low or normal

164
Q

Rising dsDNA may be a sign of

A

lupus flare

165
Q

Drug class of Terbutaline and MOA

A

Betamimetic, increase cAMP which causes a decrease in intracellular calcium leading to smooth muscle relaxation

166
Q

What femur length:foot length ratio suggests skeletal dysplasia

A

<1

167
Q

Measurements in skeletal dysplasia that suggest lethality

A

Femur length: AC <0.16

Chest circumference: AC <0.8

168
Q

Skeletal dysplasia with absent scapula

A

Campomelic dysplasia

169
Q

Dysplasia with absent or hypoplastic clavicles

A

Cleidocranial dysplasia

170
Q

Main characteristics of achondrogenesis

A
  1. severe micromelia
  2. unossified spine
  3. short trunk w/ large head

lethal

171
Q

Most common heritable, nonlethal skeletal dysplasia

A

Achondroplasia

172
Q

Main manifestations of achondroplasia

A
  1. Rhizomelia
  2. Frontal bossing
  3. Midface hypoplasia
  4. Short digits w/ Trident hand
173
Q

Disorders associated with FGFR3 mutations

A

Achondroplasia
Hypochondroplasia
Thanatophoric dysplasia
SADDAN (severe achondroplasia with developmental delay and acanthosis nigricans)

174
Q

Cases of recurrent tetra-amelia (missing all 4 limbs) has occured _____

A

in consanguineous families

175
Q

What is Roberts syndrome

A

AR – associated with tetraphocomelia and facial clefts

Cytogenetic analysis shows centrometric separation or “puffing” – pathognomic

176
Q

Main characteristics of campomelic dysplasia

A
  1. bowed femur/tibiae
  2. hypoplastic scapula
  3. disorder of sex development

Most are lethal

177
Q

Main characteristics of cleidocranial dysplasia

A
  1. wide cranial sutures
  2. hypomineralization of skull
  3. absent clavicles
  4. dental abnormalities
178
Q

Main characteristics of hyphosphatasia

A
  1. micromelia

2. undermineralization (moth eaten appearance)

179
Q

Main characteristics of OI

A
  1. fetal fractures – bone angulation, crumpled appearance, beading of ribs
  2. bone fragility - compression of skull with US probe
  3. wormian bones
180
Q

OI type that is perinatally lethal

A

Type II

181
Q

Short rib-polydactyly syndromes are due to what underlying pathology

A

primary ciliary dyskinesia

182
Q

Skeletal dysplasia that can be associated with echogenic dysplastic kidneys

A

Short rib-polydactyly syndromes

183
Q

Main characteristics of Thanatophoric dysplasia

A
  1. Micromelia with bowing
  2. Telephone receiver femur (type I)
  3. Cloverleaf skull (type II)
  4. Macrocephaly
  5. Trident shaped hands
  6. Small chest
184
Q

T/F: clubfoot more likely to be bilateral

A

True, 60-70%

185
Q

Genetic associations with Rocker-Bottom feet (most–> lest)

A

T18 > T13 > T15 (rare)

186
Q

Main characteristics of radial ray malformation

A
  1. absent radius
  2. radial deviation of hand
  3. absent or abnormal thumb
187
Q

Differential dx of radial ray malformations (6)

A
  1. T18
  2. Holt-Oram
  3. TAR
  4. Fanconi anemia
  5. Valproate syndrome
  6. VATER/VACTERL
188
Q

Holt-Oram main findings

A
  1. Cardiac septal defects

2. Radial ray malformation

189
Q

Main characteristics of Meckel-Gruber Syndrome

A
  1. Cystic renal dysplasia
  2. postaxial polydactyly
  3. posterior encephalocele
190
Q

Most common aneuploidy with polydactyly

A

T13

191
Q

Associated with pre-axial polydactyly

A

Diabetic embryopathy

192
Q

Most common aneploidy associated with arthrogryposis

A

T18

193
Q

Most common sign/sx of arthrogryposis

A

lack of fetal movement

abnormal extremity position on ultrasound

194
Q

Dysplasias to think of with curved/angulated bones (5)

A
  1. OI
  2. Thanatophoric dysplasia
  3. Campomelic dysplasia
  4. Hypophosphatasia
  5. Diabetic embryopathy
195
Q

Dysplasia to think of with abnormal ossification

A
  1. OI
  2. Arthrogryposes, akinesia sequence
  3. Achondrogenesis
  4. Hypophosphatasia
196
Q

How are the fetal o2 requirements met

A
  1. increased maternal blood supply to placenta
  2. increased fetal blood supply to placenta
  3. Fetal Hgb has higher O2 affinity than maternal Hgb
  4. Higher Hgb concentration in fetus
  5. Double Bohr effect
197
Q

% of neonatal HgF at term

A

80% of Hgb is HgbF

198
Q

What does double Bohr effect mean – ie how are the curves moving

A

The dissociation curve is happening in maternal and fetal circulations and moving opposite directions

199
Q

pO2 for uterine and umbilical vessels

A

Uterine artery: 100mm Hg
Uterine vein: 50mmg Hg

Umbilical vein: 28mmg Hg
Umbilical artery: 18mm Hg

200
Q

sO2 for uterine and umbilical vessels

A

Uterine artery: 98%
Uterine vein: 75%

Umbilical vein: 70%
Umbilical artery: 45%

201
Q

pCO2 for uterine and umbilical vessels

A

Uterine artery: 32mmHg
Uterine vein: 45mmHg

Umbilical vein: 40mmHg
Umbilical artery: 50mm Hg

202
Q

T/F: Progesterone levels can remain elevated for weeks following demise

A

True

203
Q

Source of elevated maternal and fetal deoxycorticosterone levels

A

it is a metabolite of progesterone

204
Q

Where is estrogen produced <7 weeks and > 7 weeks

A

CL until about 7 weeks then placenta takes over

205
Q

where is most fetal plasma cholesterol made

A

de novo in fetal liver

206
Q

does the fetus have high or low LDL plasma levels

A

Low — being used up by adrenal gland

207
Q

cause of elevated estrogen levels

A

hemolytic disease of newborn

208
Q

What has higher risk of maternal mortality — CHD or valvular heart disease

A

valvular heart disease

209
Q

Valvular disease that carries the biggest potential risk in pregnancy

A

mitral stenosis

210
Q

Inotropic medication that can directly decrease uterine blood flow

A

Dopamine (but paradoxically may increase flow if it improves maternal parameters)

211
Q

Potential complication of severe pulmonary stenosis

A

right heart failure

212
Q

4 things to avoid in mitral stenosis management

A
  1. tachycardia
  2. decreased SVR/hypotension
  3. acute increased preload
  4. increased pulmonary pressure (hypoxia)
213
Q

If unrepaired ASD/VSD/PDA what are some things to avoid

A

anything that increase the left to right shunt

  • -systemic hypertension
  • -decreased pulmonary vasular resistance
  • -SVT
214
Q

What events cause increased pulmonary vascular resistance

A

Hypoxemia
Hypercarbia
Metabolic acidosis
Excess catecholamines

215
Q

Patient population at increased risk of intracranial aneurysms

A

Aortic coarctation – 10%

216
Q

Complications in uncorrected coarctation

A

Hypertension
Coronary artery disease
Aortic dissection
Heart failure

217
Q

Findings of TOF

A
  1. VSD
  2. Overriding aorta
  3. RVOT obstruction
  4. RVH
218
Q

Most common cause of death with TOF

A

Sudden cardiac arrest and heart failure

219
Q

Aortic root diameter in Marfans that is an indication for preconception repair

A

> 45mm

220
Q

3 major risk factors of MI

A

age >30
HTN
DM

221
Q

when in respiratory cycle to take swan-ganz catheter measurements

A

end of expiration

222
Q

Steroid enzyme absent in the fetus

A

3beta-OHSD

223
Q

CAH is most commonly caused by defect in what enzyme

A

21-alpha hydroxylase

224
Q

CAH leads to an increase in….

A

17OHP and androgens

225
Q

Treatment for CAH in mother….CAH in fetus

A

Maternal - give hydrocortisone

Fetal - give dexamethasone starting at 7-8weeks, continue if female fetus

226
Q

Supplements for women s/p bariatric surgery

A 
B12
Folate
Iron
Vit D
Calcium
227
Q

For cord blood gas, if left out for >20 minutes, will the pH be falsely low or falsely high

A

falsely low (falls 0.05 after 30 minutes)

228
Q

Normal UA blood gas

A

pH - 7.27
pCO2 - 50
HCO - 22
Base excess - -2.7

229
Q

UA blood gas <____ considered acidosis

A

7.0

230
Q

UA cord gas, how to distinguish metabolic and respiratory acidosis

A

Metabolic - low pH, low HCO3

Respiratory - low pH, normal HCO3

231
Q

UA base deficit associated with adverse neonatal outcomes

A

> /= 12

232
Q

Most common heart defect in DiGeorge

A

TOF

233
Q

What causes volume exapnsion in pregnancy

A

peripheral vasodilation leads to activation of the renin-angiotensin-aldosterone system

234
Q

Maternal/paternal risk of passing on balanced translocation

A

Maternal - 10-15%

Paternal - 1-2%

235
Q

what would an affected offspring of this parent be: 45, XX, der (21;22)

A

46, XX, der (21;22), +21

236
Q

Microcytic anemia with High HgbA2

A

B-thal

237
Q

Swan parameters that increase in pregnancy

A

CO, SV, HR

238
Q

Swan parameters that decrease in pregnancy

A

SVR, PVR, colloid osmotic pressure

239
Q

Swan parameters with no significant change

A

PCWP, central venous pressure, mean arterial pressure

240
Q

Respiratory parameters that decrease in pregnancy

A

TLC
FRC
ERV
RV

241
Q

Respiratory parameters that increase in pregnancy

A

TV

IRV

242
Q

Respiratory parameters with no change in pregnancy

A

Vital capacity

243
Q

Sequence of loss of parameters in BPP

A 
Breathing
FHR accels
Movement
Tone
Fluid
244
Q

How does glucose cross the placenta

A

facilitated diffusion

245
Q

How do Vitamins A/D/E/K cross placenta

A

simple diffusion

246
Q

Typical fetal heart rates in cases of fetal SVT and fetal atrial flutter

A

SVT: 220-300

Atrial flutter: 350-500

247
Q

Recommended diet with diabetes

A

3 meals and 3 snacks

248
Q

Caloric intake for diabetic women — normal weight, obese weight

A

Normal - 30kcal/kg
Obese - 25kcal/kg

in general 2000-2400kcal

249
Q

Diet composition for diabetics

A

Carbs - 40-50%
Protein - 20%
Fat - 30-40% (<10% saturated)

250
Q

What epigenetic modification turns genes “off”

A

methylation

251
Q

Define imprinting

A

Expression of a specific gene depends on the sex of the parent donating the gene

252
Q

Cause of Prader-Willi Syndrome

A

Loss of the paternal copy of a critical region on Chromosome 15 (maternal uniparental disomy)

253
Q

Cause of Angelmans syndrome

A

Loss of the maternal copy of a critical region on Chromosome 15 (paternal uniparental disomy)

254
Q

Most likely cause of imprinting and risk of recurrence

A 
random deletion (mostly)
<1% recurrence in siblings
255
Q

Chromosome abnormalities can be either numerical or _____

A

structural

256
Q

Most common chromosomal structural abnormalities

A

Translocations
Deletions
Inversions

257
Q

What do the p-arms of acrocentric chromosomes contain

A

chromosomal satellites and code for ribosomal RNA’s

258
Q

of chromosomes in a balanced translocation

A

45

259
Q

Average gene density

A

1 gene pere 50 kilobases

260
Q

How do chromosome inversions happen

A

2 breaks on same chromosome – the intervening segment rotates and reintegrates in an upside down position

261
Q

Difference between paracentric and pericentric inversions

A

paracenteric - the inverted chromosome segment is located on one side of the centromere (ie only 1 arm), usually associated with infertility/miscarriages

pericentric - the inverted segment involves both chromosome arms and spans the centromere, increased risk of phenotypic abnormalities in offspring

262
Q

Definition of macrodeletion

A

3-5 megabases

263
Q

CAH leads to increased levels of what two hormones

A

17-OHP and androgens

264
Q

What changes in oxyhemoblobin curves occur in mother and fetus

A

Mother - right shift favoring release of O2 at any pO2

Fetus - left shift favoring high saturation at any pO2

265
Q

What is the effect of 2,3-BPG on adult hemoglobin

A

Decreases the ability of adult hemoglobin to bind O2 (decreased O2 affinity)

266
Q

T/F: fetal hemoglobin is unaffected by 2,3-BPG

A

True

267
Q

X & Y axis of Bohr model

A

X axis: pO2

y axis: % hemoglobin saturation

268
Q

At the same pO2 does the fetus or mother have higher oxygen saturation

A

fetal

269
Q

What is the Bohr effect

A

The ability of CO2 to effect the oxygen affinity of hemoglobin

270
Q

Bohr effect in mother

A

Increase CO2 in mother from fetal offloading –> hemoglobin more likely to release oxygen

271
Q

Bohr effect in fetus

A

Decreasing CO2 due to offloading to mother –> higher hemoglobin affinity for O2

272
Q

3 things that favor movement of oxygen from maternal to fetal compartment

A
  1. difference in pO2, creates gradient favoring fetal movement
  2. fetal hemoglobin with higher affinity for oxygen than maternal hemoglobin
  3. double bohr effect
273
Q

What factors can cause a more right-shift of maternal oxyhemoglobin curve

A

High DPG
High temp
Low pH (acidosis)

274
Q

Coagulation factors increased in pregnancy

A

Factors 7, 8, 9, 10, 12
vWF
Fibrinogen

275
Q

Coagulation factors without change in pregnacny

A

Prothrombin

Factor 5

276
Q

DVT more likely in what leg

A

left

277
Q

Function of Protein C and S

A

To prevent clot from propagating to normal endothelial tissue

278
Q

Molecule in clotting cascade the cleaves fibrin

A

Plasmin

279
Q

PT/PTT which clotting pathway is being tested

A

PT - extrinsic

PTT - intrinsic

280
Q

Factors in the intrinsic cascade

A

8, 9, 11, 12, PLTS

281
Q

Clotting cascade, common pathway factors

A

Calcium, 5, 10

282
Q

Dermatomal level needed for c-section

A

T4

283
Q

At what CRL should midgut herniation not be seen

A

after 54mm it is suspicious

after 61mm definitely abnormal

284
Q

Time period in GA for ONTD exposure

A

21-28 days after conception

285
Q

urogenital anomalies, upturned upper lip, clotting issues, macrocephaly, hypoplasia of nails/digits

A

fetal hydantion syndrome

286
Q

Neonatal live vaccine in first 6 months of life, avoided with some IBD drugs

A

Rotavirus

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