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nicu boards > NICU boards cards 1 > Flashcards

NICU boards cards 1 Flashcards

1
Q

What types of shock can sepsis present as?

A

Hypovolemic or distributive

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2
Q

In hypovolemic shock, what are the changes in CO, ventricular filling, and stroke volume?

A

Ventricular filling: decreased
CO: decreased
SV: decreased

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3
Q

In distributive shock, what are the changes in circulating blood volume and ventricular filling?

A

Ventricular filling: decreased
Normal circulating blood volume

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4
Q

What type of heart sound occurs with PHTN?

A

single or narrowly split, loud S2

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5
Q

What is the first and second most common cyanotic heart disease presenting in 1st week of birth?

A

1st: TGA
2nd: HLHS (this is most common cause of mortality in 1st week)

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6
Q

What is most common overall congenital heart defect? What type is most common?

A

VSD
peri membranous - muscular most likely to close

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7
Q

What is most common cyanotic heart disease beyond infancy?

A

Tetralogy of Fallot

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8
Q

What are the L to R shunt congenital heart defects?

A

VSD, PDA, ASD, complete AV canal, Partial anomalous pulmonary venous return

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9
Q

What is L-TGA?

A
  • congenitally corrected heart disease, «1% of all congenital heart disease
  • aortic valve is to LEFT (hence L) of pulmonary valve
  • no need to have shunt between 2 circulations
  • high assoc w other cardiac defects
  • acyanotic w mnimal resp distress if no other issues - but majority will have other cardiac defects so will be cyanotic
  • single S2
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10
Q

Most common type of prenatal poor tracing?

A

Variables

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11
Q

With what cardiac anatomy do you have reversed differential cyanosis?

A

D-TGA with PDA, intact ventricular septum, and one of the following:
- PHTN
- coA
- interrupted aortic arch

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12
Q

What murmur with DTGA?

A

Loud and sigle S2
- loud bc aortic valve below sternum & anterior
- single: pulmonary valve is posterior and not heard

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13
Q

DTGA: is there CHF? What type of hypertrophy?

A

Yes CHF secondary to left sided overload
RVH, can have R atrial hypertrophy and can also have combined ventricular hypertrophy if large VSD PDA PS LVOT obstruction
Has R QRS axis

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14
Q

What type of VSD is found in TGA?

A

Typically perimembranous, always large and unrestrictive, so LVP equal RVP’s

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15
Q

Murmur with tetralogy?

A

PS murmur only if small, VSD murmur if pink tet (since effect of L->R VSD shunt will be more minimal), otherwise S2 often single and loud (because aorta is anterior when overriding)

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16
Q

Murmur with pulmonary atresia with intact ventricular septum?

A

Single s2, no other murmur

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17
Q

EKG with pulmonary atresia with intact ventricular septum?

A

Normal QRS axis
(TA has a superior/R axis)
LVH, RVH since RV is hypoplastic and hypertrophied in most cases

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18
Q

How does mixing occur with pulmonary atresia with intact ventricular septum?

A

Requires ASD/PFO with PDA to survive

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19
Q

How does mixing occur with truncus arteriosis?

A

Large VSD ALWAYS present

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20
Q

What kind of pulse pressure with truncus arteriosis?

A

Wide pulse pressure and bounding pulses from diastolic runoff in PA

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21
Q

EKG findings with tricuspid atresia

A

L axis deviation

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22
Q

Ebstein’s anomaly - what types of shunts?

A

80/90% have ASD with R to L shunt
Also requires PDA with L to R flow for adequate pulmonary blood flow

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23
Q

EKG findings with Ebstein’s

A

RBBB, WPW, RA enlargement, occasional 1st degree AV block

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24
Q

What other anomalies is single ventricle associated with?

A

Higher risk of asplenia or polysplenia

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25
Q

What causes a single S2?

A
  • When one valve is absent, like in pulmonary atresia or truncus arteriosis
  • or when both valves close at same time due to equal ventricular pressures, like double outlet single ventricle or VSD with equal ventricular pressures
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26
Q

What shunts are required for systemic oxygenated blood flow with TAPVR?

A

ASD or PFO (R to L shunt)

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27
Q

Types of TAPVR?

A

Supracardiac (most common) - PV to SVC via innominate, azygous, or direct
Cardiac - PV into RA directly (or indirectly via coronary sinus)
Infracardiac/Subdiaphragmatic - PV crosses diaphragm, drains into portal/hepatic vein or IVC
Mixed

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28
Q

Presenting sxs of obstructive TAPVR

A

Majority subdiaphragmatic/infracardiac are obstructive
- cyanosis
- resp distress
- decreased systemic perfusion

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29
Q

Presenting sxs of nonobstructive TAPVR

A

Majority cardiac or supracardiac are nonobstructive
- mild to mod cyanosis (depending on degree of mixing across ASD)
- wildely split and fixed S2 - quadruple rhythm

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30
Q

Most common cause CHF after 2nd week of age?

A

VSD

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31
Q

What are the constrictors of PDA?

A

O2, PGF2a, acetylcholine, bradykinin

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32
Q

What are the dilators of PDA?

A

PGE1, PGI2 (prostacyclin), hypoxemia, acidosis

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33
Q

What is the ekg finding with complete av canal?

A

Left superior (left atrial) QRS axis bc of posterior and inferior rotation of AV node and anatomical abnormality of HIS bundle. Often has 1st degree AV block/prolonged PR
RVH, +/- LVH

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34
Q

is PAPVR symptomatic?

A

Often asymptomatic, usually ASD murmur with fixed split S2, can have increased heart size, amt of pulm blood flow dependent on # of anomalous pulm veins, ASD size and presence, and degree of PVR

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35
Q

1 cause of late onset sepsis

A

coagulase negative Staph

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36
Q

EOS has greater risk of what organ involvement? LOS?

A

EOS: pneumonia
LOS: meningitis

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37
Q

Most common 2 organisms causing EOS?

A
  1. GBS, 2. E.coli
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38
Q

Most common site of origin of osteomyelitis?

A

Metaphysis

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39
Q

Most common serotype of GBS infx?

A

serotype 3

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40
Q

Most common bones for osteomyelitis?

A

Femur>hyumerus>tibia>radius>maxilla

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41
Q

Etiologies of osteomyelitis or septic arthritis (HIGH YIELD)

A
  1. hematogenous spread s/p bacteremia
  2. directly puncture wound
  3. spread from adjacent infx
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42
Q

Most common E.coli antigen leading to meningitis?

A

K1 antigen

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43
Q

When is onset of chemical conjunctivitis?

A

within 24h of exposure

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44
Q

Which is earlier timing of onset, gonorrhea or chlamydia conjuntivitis?

A

Gonorrhea, 2-5 days of age

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45
Q

What is the most common cause of conjunctivitis in month 1 of age?

A

Chlamydia (much more common than gonorrhea)

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46
Q

How to diagnose chlamydia conjunctivitis?

A

Giemsa stain of conjunctival scrapings

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47
Q

How to treat gonorrhea conjunctivitis?

A

3rd generation cephalosporin
(prevent with prophylaxis - prophylaxis does not completely prevent chlamydia conjunctivitis)

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48
Q

How to treat chlamydia conjuncivitis?

A

oral erythromycin x 14 days

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49
Q

Which is worse - gonorrhea or chlamydia conjunctivitis?

A

Gonorrhea - No Good - medical emergency, can progress to involve cornea and ulceration/perforation

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50
Q

What type of bacteria is GBS

A

gram positive dipococci in chains and pairs, catalase neg

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51
Q

What type of bacteria is Listeria

A

Gram positive rod

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52
Q

What type of amniotic fluid w Listeria?

A

chocolate colored or meconium-like stained amniotic fluid

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53
Q

Why do brain imaging of Listeria meningitis?

A

Brain abscess, which would prolong tx course

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54
Q

what type of bacteria is Neisseria gonorrhea?

A

Gram negative intracellular diplococci in pairs

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55
Q

What to do in asymptomatic neonate born to mother with untreated gonorrhea?

A

1 dose ceftriaxone

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56
Q

What type of bacteria is chlamydia?

A

obligate intracellular bacteria - not detectable by gram stain

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57
Q

What to do in asymptomatic neonate born to mother with untreated chlamydia?

A

Nothing until infant develops clinical signs of infection; efficacy of prophylaxis is unknown

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58
Q

How is syphilis transmitted?

A

Majority transplacental, can be contact w active lesion at delivery

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59
Q

how is congenital TB spread?

A

It is rare but
1. hematogenous via placenta
2 ingestion of infected amniotic fluid

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60
Q

Gram stain/type of bacteria of bordatella pertussis?

A

gram negative pleomorphic bacillus

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61
Q

CBC findings in pertussis?

A

lymphocytosis

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62
Q

Type of bacteria of clostridium botulinum

A

anaerobe gram positive bacillus

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63
Q

Diagnosis of clostridium botulinum?

A

detect toxin in stool or stool culture

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64
Q

Pathophysiology of how c. botulinum works?

A

toxin inhibits release of Ach from nerves

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65
Q

How to treat c. botulinum?

A

human derived IV botulism immune globulin

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66
Q

Type of bacteria of clostridium tetani?

A

gram positive bacillus

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67
Q

Pathophysiology of how c. tetani works?

A

releases tetanus toxin that blocks gaba release in neuromuscular junction

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68
Q

Treatment of c. tetani?

A

tetanus immune globulin to neutralize circulating toxin and parenteral Pen G 0-14 days

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69
Q

Type of bacteria of ureaplasma urealyticum?

A

small pleomorphic bacteria that lack a cell wall

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70
Q

Type of bacteria of H flu?

A

gram negative coccobacillus, both encapuslated or unencapsulated/nontypeable (most common)

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71
Q

What should be given in H flu meningitis?

A

dexamethasone to decraese risk of hearing loss before or with first dose abx

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72
Q

what does vitamin A do?

A

pulmonary epithelial growth & cellular differentiaion
may play role in CLD,
deficiency: photophobia, scaling, abnl epiphyseal bone formation and tooth enamel

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73
Q

B1 thiamine imp for?

A

Beri Beri - “B” like blueberry very round like cardiac failure and constipation, fatigue, irritability
assoc w pyruvate dehydrogenase complex def iciency and maple syrup urine disease

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74
Q

B2 riboflavin imp for?

A

B2 is like seeing double - blurry vision. dermatitis and mucositis, assoc with glutaric aciduria type 1

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75
Q

B6 pyridoxine

A

hypochromic ANEMIA, dermatitis, mucositis, seizures, homocystinuria “homoSIXtinuria”

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76
Q

B7 Biotin

A

alopecia, dermatitis, scaling, seborrhea, assoc w biotinidase deficiency, propionic acidemia, and the most metabolic derangements

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77
Q

Vitamin C

A

scurvy, poor wound healing, bleeding gums, assoc w transient tyrisonemia “TANGERINES which are CITRUS”

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78
Q

Holder pasteurization destroys what in donor milk?

A

lymphocytes, alk phos, lipoprotein, some lipases, Immunoglobulin M all Melted, cytokines, growth factors, lactoferrin reduced by 50%, lysozyme reduced by 25%
what is preserved - most of IgA and IgG, oligosaccharides, vitamins ADE (fat soluble), lactose, long chain polyunsaturated FAs LPUFAs, epidermal growth factor

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79
Q

What does microwaving milk reduce?

A

IgA, all immunoglobuins, lysozyme, and vitamin C. Nothing else is stable with microwaving

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80
Q

Hindmilk contains - more/less protein, fat, lactose?

A

More fat, less lactose, same protein,

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81
Q

Most common cause of neonatal late onset UTI?

A

Ecoli

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82
Q

Treatment of UTI in neonates

A

amp/aminoglycoside, should be IV due to high assoc of sepsis with UTI

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83
Q

function of sertoli cells in sexual differentiation?

A

regression of mullerian duct to steer fetus toward male dev

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84
Q

function of leydig cells?

A

testicular development and wolffian duct differentiation. Leydig cells are LIT (produce INS3 & L3 -> testicular, testosterone -> wolffian duct)

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85
Q

Wolffian ducts develop into?

A

epididymis, vas defersn, ejaculaltory duct, seminal vesicles

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86
Q

SRY gene function?

A

lead to differentiation of gonads to testes

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87
Q

What does 5a reductase do?

A

convert testosterone to DHT.

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88
Q

Most common cause of hypertension in neonates?

A

renal artery thrombosis

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89
Q

Anatomic dead space

A

lung areas not involved in gas exchange

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90
Q

Alveolar dead space

A

alveoli not involved in gas exchange with vasculature (V/Q>1)

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91
Q

physiologic dead space

A

anatomic + alveolar dead space, or wasted ventilation. physiologic dead space is always greater than anatomic dead space

92
Q

Embryonic phase lung dev

A

0-5w, resp tract forms from endoderm**, lung forms from ventral bud of esophagus,
pulm blood vessels and smooth muscles from mesoderm

93
Q

Pseudoglandular

A

fetal lung fluid begins to form, separation of thorax and peritoneal cavity, onrespiratory bronchioles form/all large airways up to terminal bronchi established, vasculature begin to est

94
Q

Canalicular

A

~15-25w, respiratory bronchioles form - distal epithelium/pneumocytes begin to differentiate (type II pneumocytes vegin to differentiate into type I), units of gas exchange begin to develop - lamellar bodies begin to appear and store surfactant, surfactant production begins ~24w, at this time there is an adult # of airways (bronchi, etc all complete)

95
Q

Saccular stage

A

~25-35w, last generation of air spaces, multiple sacs beginning to form from terminal bronchioles. At 32w you have enough surfactant to prevent atelectasis, the surface of gas exchange matures into alveolar ducts. capillaries invade alveolar membrane

96
Q

Alveolarization

A

36w - age 3 to 8y, increase in bronchioles and alveoli mature from alveolar sacs, microvascular growth and vessel maturation

97
Q

What substances enhance lung development?

A

thyroxine, vitamin A

98
Q

What delays alveolarization?

A

postnatal steroids, oxygen, nutritional deficiencies, mech ventilation, insulin, inflammation

99
Q

What issues occur in embryonic phase of lung dev?

A

(ventral bud forms from esophagus - things are supposed to separate)
laryngeal clefts, TEF, bronchogenic cysts

100
Q

What issues occur in pseudoglandular phase of lung dev?

A

***congenital diaphragmatic hernias, branching abnormalities, congenital lobar emphysema, cystic malformations, pulmonary lymphangiectasias

101
Q

What issues occur in canalicular phase of lung dev?

A

surfactant deficiencies, pulmoary hypoplasia, alvolar CAPILLARy dysplasia

102
Q

What issues occur in alveolarizaion phase of lung dev?

A

vasculature is maturing - pulmonary hypertension

103
Q

Most physiologically active componnt is

A

DPPC 50% phosphatidylcholine, overall 70-80% phospholipid
10% surfactant, 10% cholesterol

104
Q

type I pneumocytes shape

A

long flat thin, make up majority of surface area but there are fewer in number, gas exchange bt alveoli and capillaries

105
Q

type II pneumocytes shape

A

chunkier (number II is chunkier than type I)

106
Q

What inactivates surfactant?

A

meconium asp, pulm hemorrhage, pneumonia/sepsis

107
Q

How does meconium aspiration inactivate surfactant

A

phospholipase A2 in meconium competitively displaces surfactant from alveoli

108
Q

How does pulmonary hemorrhage inactivate surfactant?

A

neutrophils released following endothelial damage from blood -> free radicals that directly damage type II cells

109
Q

Which surfactants critical for surfactant function?

A

SP-B and SP-C

110
Q

Bartter’s syndrome?

A

polyhydramnios, triangular eyes, strabismus, hyponatremia (severe salt wasting, treat by taking salt tblets), hypochloremia, hypokalemia, metabolic alkalosis, hypercalciuria (**get this since Na Cl and K not being reabsorbed and you have to balance the anions that build up)

111
Q

What is urine production rate in a 20w infant and term infant?

A

5ml/h at 20w and 50ml/h at term gestation

112
Q

What branchial arch does ductus arteriosus form from?

A

6th

113
Q

How many nephrons at birth?

A

1 million (at 34w)

114
Q

Why can’t neonates concentrate urine?

A

Insensitivity to vasopressin, short loop of henle (which usually reabsorbs), low osmolality of medullary interstitium, low serum urea

115
Q

What moves oxyhemoglobin dissociation curve to left?

A

LOW (low acidity, low paCO2, low 2,3 DPG, low temp

116
Q

What is physiologic dead space?

A

alveoli + anatomic dead space.

117
Q

what is imp for pulm epithelial growth and cellular differentiation?

A

vitamin A.

118
Q

Vitamin D

A

assoc w rickets, FTT, poss tetany

119
Q

Hind milk?

A

high fat, equal protein, less lactose (body wants baby to get more simple sugars first)

120
Q

What % of caloric content is fat responsible for in breastmilk?

A

Fat responsible for 50% of caloric content of breast milk

121
Q

Do triglycerides or cholesterol vary by diet and age in breastmilk?

A

triglycerides

122
Q

Chromium?

A

regulates glucose levels. CHROME is shiny like hersheys kisses

123
Q

Copper?

A

Anemia & neutropenia, depigmentation of hair/skin, ataxia & hypotoni. Copper penny - critical for production of red blood cells and absorption of iron

124
Q

Manganese?

A

Enzyme activation of superoxide dysmutase

125
Q

Selenium?

A

Cofacter for glutamine peroxidase, deficiency is cardiomyopathy. Selena broke your heart

126
Q

11 beta hydroxylase deficiency?

A

Elevated testosterone, cortisol deficiency, Do not need to replace mineralocorticoids because DOC are very good mineralicorticoids. No salt wasting, able to retain sodium. Can present with hypertension. Still need to replace glucocorticoid
2nd most common common

127
Q

Erb’s palsy nerve roots

A

C5 & c6 (sometimes c7)

128
Q

bitemporal wasting, hypotonia, poor suck with poor feeding, poor weightgain, excessive sleepiness, almond shaped eyes, narrow nasal bridge, small hands and feet, lightly pigmented skinhair, cryptorchidism small phallus, small labia minora and clitorus

A

Prader willi neonatal presentation

129
Q

Abnormal methylation studies c/w?

A

prader willi. All due to imprinting/methylation on 15q11. 75% of cases caused by microdeletion of paternally inherited 15q11 OR 15-20% of ases from uniparental disomy where both copies of chromosomal region inherited from the mother

130
Q

CTG trinucleotide repeat expansion in DMPK

A

myotonic dystrophy DM1 (autosomal dominant, hypotonia, resp failure, feeding difficulty, mothers with stiff handshake/grip and myopathic facies

131
Q

Elevated creatine kinase, hypotonia, weakness, resp insufficiency, hypertrophic cardiomyopathy, feeding difficulty, macroglossia, hepatomegaly, hearing deficits

A

Pompe disease/glycogen storage disease type II. Deficiency of alpha glucosidase

132
Q

Hirschsprung’s is a defect in what?

A

Lack of ganglion cells, 80% rectosigmoid,

133
Q

What opens PDA?

A

acidosis/sepsis, prostacyclin, prostaglandin E1

134
Q

What closes PDA?

A

Oxygen, prostaglandin f2a, acetylcholine, bradycholine

135
Q

Atrial flutter - pattern, treatment

A

multiple sawtooth p waves after QRS.
treatment is waiting, then cardioversion or esophageal pacing if unstable. Long term digoxin. No ice to face bc atrial flutter doesnt involve AV node

136
Q

What part from embryology is invovled in renal agenesis?

A

ureteric bud

137
Q

tuberous sclerosis

A

Ash leaf spots, enamel pits and teeth, cardiac rhabdomyomas, CNS tumors, eye involvement, seizures, mental deficiencies, renal aomalies
chromosomes 9 & 16, autos dominant

138
Q

neurofibromatosis

A

cafe au lait macules - hyperpigmented, chromosome 17, axillary freckling, macrocephay, aqueductal stensosi, neuromas, fibromas, pheochromocytomas

139
Q

Names of genetic sydromes caused by Microdeletions

A

William D Warp MD, williams syndrome, DiGeorge, WAGR, Angelman, Rubenstein Taibe, Prader Willi, MicroDeletions

140
Q

leukocyte adhesion defiicency

A

too much ADHESION of umbilical cord (delayed cord separation), abnormal neutrophil function even though they have normal amts of neutrophils, neutrophils can’t grab onto blood vessels and adhere to leave circulation, bacterial infections and poor wound healing

141
Q

chediak higashi

A

abnl neutrophil degranulation leading to abnl phagocytosis, large intracytoplasmic granules in white cells, partial oculocutaneous albinism, nystagmus, peripheral neuropathy, bacterial infx

142
Q

chronic granulomatous disease

A

X linked causing NADPH oxidase issue, increased abscesses, granulomas

143
Q

hyper IgE

A

Job’s syndrome, abnl chemotaxis (pay TAXES for your job), E for eczema, coarse facial features

144
Q

Kostmann syndrome

A

german Krig (K kostman, R= autos recessive, I=infxs, G=treat w GCSF), severe congenital neutropenia (anc<500), first few months of life lots of bacterial infx like utis abscesses, developmental delay and myelodysplsatic syndrome

145
Q

buzzwords with Listeria

A

chocolate colored or meconium-like stained amniotic fluid, microabscessess, tend to be pneumonia early and meningitis late

146
Q

Most common blood vessel affected in neonatal stroke

A

left MCA

147
Q

Syndromes with glaucoma

A

Sturge Weber, trisomy 21, NF, congenital rubella, stickler syndrome, long term corticosteroid use, retinoblastoma

148
Q

Sturge Weber

A

glaucoma, port wine stain - if includes forehead eye maxillary area there is 50% chance of glaucoma, seizures

149
Q

Beckwith Wiedemann - features, chromosome?

A

Hemihypertrophy, LGA, macroglossia, hypoglycemia, ear pits or tags, omphalocele,cardiomyopathy, wilms tumor, hepatoblastoma, wilm’s tumor, exopthalmos, malrotation
Chromsome 11

150
Q

Hurler syndrome

A

corneal clouding, mucopolysaccharidoses/lysosomal storage disease, skeletal abnormalities (the HURLER who throws), heart disease, resp problems, deafness

151
Q

Leukocoria - what is most common cause and congenital infx associated?

A

Most common are cataracts (also retinal detachment, hemorrhage).
Rubella

152
Q

Hyoptonia and undescended testes

A

PWS

153
Q

Prader Willi

A

almond shaped eyes, hypotonia, poor feeding, small hands and feet, bitemporal wasting/low muscle bulk
Uniparental disomy - receives both copies from mother

154
Q

Angelman syndrome

A

Happy puppet -jerky movements/ataxia, happy demeanor, hypotonia, mental deficiencies, seizures, protruding tongue, maxillary hypoplasia,

155
Q

disorders of glycosylation

A

hypotonia, failure to thrive, strabisumus, cardiomyopthy, hypoglycemia, seizures

156
Q

myotonic dystrophy

A

hypotonia in skeletal and cardiac muscle, lots resp failure, feeding difficulty, generalized muscle weakness, contractures, masklike facies, mothers also with mytotonic facies firm handshake
trinucleotide CTG repeats***, worse with repeat generations

157
Q

SMA

A

anterior horn issues, motor delays, early death from respiratory compromise, muscle fasciculations, generalized weakness, joint contractures, absent deep tendon reflexes
SMN genes

158
Q

Preterm formula whey:casein?

A

60:40 to 80:20 range, mature breast milk about 55:45

159
Q

Copper deficiency

A

Anemia (copper is red like a penny which looks like rbc), neutropenia, osteopenia
Menke’s disease - x linked disorder with severe Cu deficiency

160
Q

Drugs that are absolute contraindications to breastfeeding

A

illicit drugs where regimen not being followed/controlled

161
Q

Vitamin E deficiency

A

hemolysis, anemia, reticulocytosis with giving iron. Given with epo (Epogen starts with E)

162
Q

Vitamin B12

A

macrocytic anemia

163
Q

Preterm breast milk vs term milk

A

Less lactose, more Na Cl, less cholesterol, more long chain PUFAs, more protein

164
Q

Kcal/g in fat?

A

9

165
Q

kcal/g in carbs?

A

3.4

166
Q

kcal/g in protein?

A

4 (like someone flexing bicep)

167
Q

In tpn cholestasis, what to reduce and what to increase in tpn?

A

decrease Manganese Copper, Inc zinc

168
Q

In renal insufficiency, what to reduce in tpn?

A

decrease chromium & selenium

169
Q

function of jejunum?

A

absorb protein, some fat, carbs, iron Ca Mg

170
Q

fx of ileum?

A

B12, bile salts, absorb fat-soluble vitamins, can compensate somewhat for jejunal resection

171
Q

Ileocecal valve fx?

A

regultes transit time, keeps reflux of colonic bacteria

172
Q

colon fx?

A

water and electrolyte resabsorption

173
Q

placental lactogen fx?

A

plays most imp role in metabolic and nutritient of fetus from placenta, creates insulin resistance causes high glucose & lipids which then upregulates insulin. High insulin promotes adiposity. placental lactogen is regulated by insulin like growth factor

174
Q

What are the essential fatty acids?

A

linoleic and linolenic acids

175
Q

essential fatty acid deficiency?

A

dermatitis, alopecia, thrombocytpoenia, FTT, bacterial infxs
increased triene to tetraene ratio

176
Q

long chain PUFAs - omega 6 and 3 imp in brain and retinal

A
  • omega 6 and 3 imp in brain and retinal
177
Q

what meds induce p450?

A

phenobarbital, phenytoin, rifampin, dexamethasone

178
Q

what meds inhibit p450?

A

chloramphenicol, simetidine, erythromycin, fluconazole, ranitidine, omeprazole

179
Q

Caffeine mechanism of action in dec apnea of prem**

A

adenosine antagonism, decreased hypoxic ventilatory depression, enhanced diagphragmatic contractility from stimulation of Ca release, increased minute ventilation, inhibits GABA receptors

180
Q

large tongue, hepatomegaly, hypotonia, enlarged heart

A

pompe disease
elevated cpk

181
Q

deletion on chromosome 11

A

WAGR. Wilms, aniridia, GU anomalies, mental Retardation

182
Q

DiGeorge

A

CATCH 22, 22q11.2 deletion. Cardiac (conotruncal arch abnormalities, vsd, pda, tof), Abnormal facies (micrognathia), Thymic aplasia/hypo, Cleft, Hypocalcemia (from hypoplastic parathyroid glands)
deficient cellular immunity, esophageal atresia, dev delay, learning abnormalities
dx by FISH

183
Q

How do amino acids cross placenta?

A

active transport by tranporters

184
Q

How do lipid soluble compounds cross placenta?

A

Diffusion based on maternal concentrations

185
Q

How do hydrophilic compounds cross placenta?

A

Transported through transtrophoblastic channels

186
Q

how does glucose cross placenta?

A

facilitated diffusion by GLUT1

187
Q

Effect of dobutamine on CO, HR, contractility

A

higher HR, contractility, higher BP, increase in cardiac output, no change in SVR

188
Q

Ca Ph how cross placenta?

A

Active

189
Q

Fat soluble vitamins and lipids how cross placenta

A

Simple diffusion

190
Q

How do most meds cross placenta

A

Simple diffusion

191
Q

Water soluble vitamins how cross placenta?

A

Active transport

192
Q

Quad screen in trisomy 21

A

inhibin A high, bHCG high, low AFP, low estriol

193
Q

Quad screen in trisomy 18

A

all low, normal inhibit A

194
Q

Werdnig Hoffman

A

SMA type 1, anterior horn atrophy of motor units on biopsy, autos recessive, severe generalized hypotonia, frog leg position, legs >arms, proximal>distal, marked head lag, bulbar weakness (poor suck swallow, tongue fasiculations, hoarse cry), absent DTR

195
Q

ACQUIRED neonatal myasthenia gravis - what is passed from mother?
clinical presentation?

A

anti Ach receptor antibodies
most require anticholinesterase therapy
in utero or at birth - poor resp failure, inbility to swallow or handle secretions, weak cry
duration of illness 2 weeks

196
Q

CONGENITAL myasthenia gravis

A

due to genetic defect - either autos recessive or familial infantile myasthenia (rare)

197
Q

Duodenal atresia- what embryonic process and where in duodenum does it occur?

A

2nd part of duodenum and failure of recannulization in 2nd mo of gestation

198
Q

Which values decrease with increase GA and BW? total BW, extracellular water, intracellular water, Na, Cl

A

Decrease: total BW, extracellular, Na, Cl
Increase: intracelluluar

199
Q

Human colostrum vs cow’s milk - levels of whey:casein, Na, K, Ca, Cl

A

colostrum 80:20, less Na, K, Ca, Cl, less protein, less lactalbumin
cow’s milk 20:80

200
Q

Which substances improves surfactant synthesis? estrogen, indomethacin, pge1, thyroid hormones, steroid hormones

A

all except indomethacin

201
Q

Heinz bodies

A

g6pd (small dark circles in rbcs)

202
Q

Features of beckwith wiedemann

A

umbilical hernia, lateral overgrowth, LGA, protruding tongue, nevus flammeus, b/l earlobe creases, persistent hypoglycemia
chromosome 11p15.5 in 85%

203
Q

side effects of pge1 - initial infusion and longer term?

A

initial infusion: hyperthermia, apnea, hypotension, bradycardia, seizures, apnea
later (after 9d): cortical hyperostosis, gastric outlet obstruction,

204
Q

presenttion of vitamin k deficiency

A

prolonged PT, normal platelets, normal fibrinogen

205
Q

Embryology of duodenal atresia?

A

failure of recanalizaton of intest tube in 8-10w gestation

206
Q

What part of duodenum does duodenal atresia occur?

A

2nd part

207
Q

How does intestinal atresia occur?

A

ischemia

208
Q

What parts of GI tract does intestinal atresia occur?

A
  1. distal ileum, 2) prox jejunum, 3. distal jejunum, 4. prox ileum
209
Q

Malrotation anatomy? Embryology?

A

Failure of normal rotation with abnormal fixation, assoc with narowed mesenteric variations. Small bowel shifted to R side, cecum displaced from RLQ to RUQ, presence of Ladd’s bands (fibrous bands forming bt cecum and R posterior retroduodnal peritoneum) serving as lead point around which bowel is twisted
malrotation is the risk factor for volvulus (intestinal ischemia)

210
Q

Nieman Pick

A

sPhingomyelinase (like Pick), Nieman Picks cherries

211
Q

Gaucher disease

A

Ashkenazi jewish descent, Glucocerebrosidase (starts with G), no eye abnormalities, do have hepatosplenomeg
gaucher cells in bone marrow

212
Q

Tay Sachs

A

Tay SaXs (heXosadminidase), cherry red spot

213
Q

Lysosomal storage disorders with cherry red spot

A

Niemann Picks cherries and puts them in his Sach

214
Q

surfactant B

A

predominant surfactant protein in respiraotry distress, found on chromosome 2 (B is 2nd letter of alphabet)

215
Q

LaPlace’s law

A

surfactant. P=2T/r

216
Q

Howell Jolly bodies

A

asplenia

217
Q

Heterotaxy syndrome

A

Asplenia, assoc with atrial arrhythmias
Can be due to PCD

218
Q

Downside to dopamine with prolonged use

A

Dopamine is precursor of epinephrine and decreased endogenous stores. With continued use will be less effective

219
Q

Coarctation of aorta assoc with what genetic syndrome

A

Turners. 30% of patients with Turner syndrome have coarctation of aorta

220
Q

how to test for hiv

A

hiv1 pcr 2w, 2mo, 6mo after birth

221
Q

treatment of baby born to untreated HIV pos mother

A

6w azt plus 3 doses of nevirapene

222
Q

paired t test

A

A paired t test is used for the comparison of means between 2 groups that involve the same subjects, commonly before and after an intervention, such as in the example in the vignette. As in the Student t test and other parametric tests, normal distribution of data and similar degree of variance are required for the use of the paired t test.

223
Q

Student t test

A

Student t test is used to compare the means for a numerical variable between 2 groups of unrelated study participants in which there is normal distribution of data and a similar degree of variance in both groups.

224
Q

analysis of variance (ANOVA) and Friedman tests

A

When more than 2 groups are involved, the analysis of variance (ANOVA) and Friedman tests are appropriate for normal or skewed distributions, respectively.

225
Q

χ2 test

A

The χ2 test is used to evaluate the differences in qualitative data (nominal or ordinal data) between 2 or more groups.

226
Q

rubella

A

cardiac lesions such as patent ductus arteriosus and pulmonary artery stenosis. These infants may also have myocarditis, hepatosplenomegaly, petechiae, cataracts, glaucoma, and microphthalmia.

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