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PHASE II > PEDS > Flashcards

PEDS Flashcards

1
Q

A new born with small erythematous macules/papules that become pustules on erythematous bases 3-5 days after birth

DOES NOT involve the palms or soles. Can spontaneously resolve.

A

Erythema Toxicum

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2
Q

A new born with blocked eccrine (sweat) glands that are tiny friable clear vesicles. Most commonly in neonates 0-1week old

A

Miliaria.

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3
Q

A new born with 1-2 mm pearly white/yellow papules (2/2 keratin retention in the skin) most prominent on the cheeks, forehead, or chin

A

Milia

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4
Q

6 or more of these increase the risk of NF1, especially if they are associated with axillary or inguinal freckling.

A

Cafe Au Lait Macules

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5
Q

5 diseases associated with Cafe Au Lait Spots

A

NF1
Tuberous Sclerosis
McCunne-Albright Syndrome
Bloom Syndrome
Fanconi Anemia

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6
Q

A new born with a Vascular malformation of the skin 2/2 superficial dilated dermal capillaries,

A

Port-Wine Stain treat with Pulse Dye Laster Tx in infancy for best outcomes

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7
Q

Pink/red, Sharply demarcated, blanchable macules or papules in infancy. Over time they grow and darken to purple and may develop and thickened skin appearance.

A

Port Wine Stains

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8
Q

What is the Triad of Sturge-Weber Syndrome

A
  1. Facial port wine stain (esp. Trigeminal Nerve Distribution)
  2. Leptomenigeal Angiomatosis
  3. Ocular Involvement (glaucoma)
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9
Q

What skin condition in neonates is most often seen in Asian/ East Indian/ African Americans

Is Blue/ slate grey pigmented macular lesions with indefinite borders most commonly in the presacral;/sacral/ or gluteal areas

A

Mongolian Spots, Benign- fade over years.

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10
Q

What is a pink/red irregularly shaped macular patch most commonly seen on the nape of the neck, eyelids, and forehead that typically resolves by age 2 and doesnt darken overtime.

A

Nevus Simplex ( Stork Bite)

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11
Q

What is the difference between Port Wine Stains and Stork Bites

A

Port wine Stains get darker over time, can get thickening of the skin, and can be associated with Sturgeon-Weber Syndrome,

All things that Nevus Simplex’s/ Stork bites do not.

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12
Q

Staph Scalded Skin Syndrome is most common in what age group

A

Infants (3-7 days of age) or children less than 5 yo

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13
Q

A 6 day old infancy presents with fever, increased irritability, and skin tenderness than progresses to cutaneous blanching erythema….
What is this and what is the next phase of the disease?

A

Erythema phase of Staph Skin Scalded Syndrome

Next phase is the Bullae Phase+ Nikolsky Sign.

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14
Q

How do you clinical Dx SSSS

A

Clinical Dx or Cx from blood or nasopharynx or Skin Bx

( blisters are sterile)

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15
Q

Treatment for a neonate with SSSS

A

Penicillinase Resistant PCN ( Nafcillin/ Oxacillin)

Can Add Clinda as needed.
Or Vanc if MRSA or PCN Allergy .

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16
Q

45Xo

A

Turners Syndrome

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17
Q

What are the manifestations of Turners Syndrome

A

Hypogonadism (streaked Ovaries), early ovarian failure, Primary Amenorrhea, Absent breasts, Short Stature, Webbed neck, Broad chest and nipples,

COART OF THE AORTA, MVP, HTN
Horseshoe Kidney
Hydro-Nephrosis
Hypothyroidism
DM
IBS

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18
Q

What are the Endo Labs for Turners Syndrome

A

Low E and High FSH/LH

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19
Q

What is 47XXY

A

Klienfelters Syndrome

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20
Q

What are the clinical manifestations of Klienfelters syndrome

A

The patient will appear NML until puberty onset and be very tall.

At puberty: scoliosis, language disorders, small testes scarce pubic hair, infertility

As an adult: Increased Testicular, breast, and germ cell cancers, Non Hodgkin Lymphoma

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21
Q

A male presents with MVP, hyperextendable joints, hypotonia, and macrocephaly, a LONG NARROW FACE, large ears, and enlarged testicles.

A

Fragile X syndrome

Most common gene related cause of ASD

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22
Q

What is the genome of Fragile X syndrome

A

X chromosome in the q27 regions have an expanding repeating CGG segment

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23
Q

A pt presents with prominent epicanthal folds, Brushfield spots (while spots on the iris), transverse palma crease, and AV defects (tet of fallot)

A

Trisomy 21

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24
Q

Abnormally high or low beta-hCG in a neonatal testing can indicate what genetic abNMality

A

Trisomy 21

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25
Q

In pts with Trisomy 21 will the PAPP-A be high or low

A

Low with fetal Down syndrome

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26
Q

Increased thickness of Nuchal translucency U/S can be see in what genetic abnormalities

A

Trisomy 13, 18, and 21

Test is done at 10-13 weeks.
If increased thickness is seen then test chorionic billions sampling or amonio centesis

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27
Q

What is the genetic disorder associated with skin hyperexentsibility, joint hyper mobility, and fragile connective tissues?

A

EDS ( Ehlers Danlos)

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28
Q

What genetic condition presents as smooth, velvety, fragile skin that bruises easily or may split easily with trauma, also associated with Meteniers Sign, and most common cause of death is Aneurysm Rupture?

A

Ehlers Danlos

Meteniers Sign: easy eversion of the upper eyelid)

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29
Q

What is the mutation that causes Marfan Syndrome

A

Mutation of the fibrillin-1 gene resulting in weakened connective tissue

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30
Q

What is the autosomal dominant systemic connective tissue disorder that leads to Cardio, ocular, and MSK abnormalities

A

Marfans

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31
Q

How does Marfans present

A

Cardo: MVP and progressing aortic root dilation learning to dissections and aneurysms

MSK: Tall statue, arachnodactyly, joint laxity

Ocular: ectopia lentís (dislocated lens of the eyes), myopia

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32
Q

What are the clinical manifestations of Fetal Alcohol Syndrome

A

Microcephaly, SMALL upper lip, smooth Philtrum, small palpebral fissures, and small distal phalanges.

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33
Q

What are the effects of smoking during pregnancy

A

Pre term birth, miscarriage, still birth, low Birth wt, heart defects, cleft lip/palate, malformations

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34
Q

What are the two most common neural tube defects

A

Spina Bifida and Anencephaly

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35
Q

What is the most common deficiency that leads to neural tube defects?

A

Folate

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36
Q

What is a failure of closure of the portion of the nueral tube that becomes the cerebrum

A

Anencephaly

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37
Q

What is an incomplete closure of the embryonic neural tubule that leads to non fusion of some of the vertebrae overlying the spinal cords , may present with a protrusion of the spinal cord thought the opening of the skin. Most commonly seen on the lumbar spine or sacral areas.

A

Spina Bifida

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38
Q

How do we screen for Neural Tube defects

A

Increased material serum Alpha Fetoprotein followed by amino centesis showing increased alpha-fetoprotein and increased acetylcholinesterase

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39
Q

What is the genetic disorder 2/2 a small deletion or inexpressioin of genes in the paternal copy of chromosome 15

A

Prader- Willi Syndrome

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40
Q

Genetic Disorder that presents as prenatal hypotonia, post natal growth delay, hypogonadotrphic hypogonadism, and obesity after infancy

A

Prader Willi (Chromosome 15) syndrome

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41
Q

A weak floppy baby, with cryptorchidism think

A

Prader willi syndrome

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42
Q

What is the presentation for Beckwith-Wiedmann Syndrome

A

Larger for gestational age, organomegaly, macroglossia, hypo-gl, asymmetric limbs

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43
Q

An infant with beckwith-Wiedmann syndrome is at an increased risk of what two neoplasms.

A

Hepatoblasotma and Wilms Tumor

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44
Q

A peds pt presents with ataxia, opsoclonus myoclonus syndrome, HTN, and diarrhea

A

Neuroblastoma

Most common to the adrenal medulla or para spinal region.

Opsoclonus Myoclonus Syndrome: dancing eyes and dancing feet

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45
Q

What is NF1

A

Autosomal dominant neurocutaneous d/o 2/2 mutated NF1 gene (chromosome 17) encoding for the protein neurofibromin (a tumor suppressor)

> 6 Cafe Au Lait spots
Axillary freckling
Lisch Nodues in the eyes

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46
Q

What does NF1 look like on MRI

A

Unidentified bright objects on T2 scans, in the basal ganglia, brainstem, or cerebellum.

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47
Q

What is NF2

A

An autosomal dominant d/o assoc with multiple CNS Tumors (bilateral CN VIII tumors aka schwannomas, vestibular neuromas or acoustic neuromas), spinal chords tumors, an intracranial tumors

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48
Q

What is the mutation in NF2

A

Mutation of the NF2 tumor suppressor gene (normally produces protein schwannomin aka Merlin)

49
Q

If a peds pt presents with bilateral vestibular neuromas.. think

A

NF2

50
Q

What are the optic and skin manifestations of NF2

A

Cataracts, retinal hamartomas,

Cutaneous tumors, less assoc with cafe au lait than NF1

51
Q

What medication can be used to treat hearing loss and shrink tumors in pts with NF2

A

Bevacizumab

52
Q

What is the autosomal recessive genetic d/o most common in Ashkenazi Jewish families of Eastern Europe, also seen in Cajuns on Southern Louisiana ?

A

Tay-Sachs Dz

53
Q

What is the clinical manifestation of Tay Sachs in an Infant ?

A

Increased startle reaction, loss of motor skills, decreased eye contact at 4-5 months, hyperaccusis, blindness, retardation, and dementia, that leads to SZRs by age 2 and death by 3-4

54
Q

What is the presentation of Tay-sachs in ages 2-10

A

Motor skill degeneration, dysphagia, ataxia and spasticity, death occurs between 5-15

55
Q

What is the presentation of adult onset tay-sachs

A

Unsteady spastic gait and prgressive deterioration leading to psychosis

56
Q

What are the physical exam findings of a PT with tay-sachs

A

Cherry rep spots on the macular pallor and macrocephaly

57
Q

Are live attenuated vaccines given to immunocomp or pregnant pts

A

NO!

58
Q

What is the only live vaccine that can be given to HIV pts

A

MMR, CD# must be above 200.

59
Q

What allergies are contraindicated for MMR vax

A

Neomycin and Streptomycin

60
Q

What vaccines can be given in pregnancy

A

diphtheria, tetanus, inactivated flu, HBV, rabies, menigococal

61
Q

What is an APGAR score

A

Appearance
Pulses
Grimace
Activity
Respirations

62
Q

Define prematurity

A

Before 37 weeks

63
Q

Define Late prematurity

A

Between 34-27 weeks

64
Q

Be fine moderate prematurity

A

Between 32-34 weeks

65
Q

Define very/ extreme prematurity

A

Very= before 32 weeks
Extreme= before 28 weeks

66
Q

Define LBW

A

Less than 2500g

67
Q

Define intraventricular hemorrhage on the newborn

A

Bleeding in the germinal matrix (between the caudate nucleus) w/in the 1st day of life

68
Q

A newborn on the 1st day of life presents with a bulging fontanelle, SZR, hypotonia, coma, and HOTN

Think

A

Intraventricualr Hemorrhage

Dx with Cranial U/S
-blood in the lateral ventricles
-hydrocephalus

Should be screened in all pts under 30wks

69
Q

What pts need screening for retinopathy of prematurity

A

All infants under 1500g or less than 30 wks

70
Q

How do you treat apnea of prematurity
(>85% o2 and >80bpm x 20secs)

A

Drying the baby
Rubin the back/Soles of Feet
Radiant warmer/ Incubator
Suctioning PRN
Adjust the airway
BVM PRN

71
Q

When and for what condition would you give caffine citriate to a newborn

A

Apnea of prematurity and when there are severe episodes of Hypoxemia, Cyanosis, of Bradycardia

Given as 20ml/kg bolus and then 5-50 mg/kg q24 hours

72
Q

What is a condition in neonates that causes a decrease in the # of alveoli, interstitial thickening, Pulm edema, and atelectasis

A

Broncho-pulmonary Dysplasia 2/2 Prolonged mechanical ventilation

73
Q

How do we treat Broncho-pulm dysplasia

A

Supportive tx and exploration of resp infections
Fluid restriction and diuretics

74
Q

What are the TORCH infections

A

Toxoplasmosis
Other-
Rubella
CMV
HSV-2

75
Q

What are the “other” infections of TORCH

A

Listeria
Parvovirus B19
Syphillis
Varicella

76
Q

How are TORCH infections transmitted

A

Vertically transmitted

77
Q

When is the infant at Risk of COngential toxoplasmosis

A

the 1st 2 trimesters

78
Q

A new born presents with chorioretnitis, hydrocephalus, and intracranial calcifications

What is the TORCH infection suspected?

A

Toxoplasmosis

79
Q

A baby with blue-ish marks on the skin

What TORCH infection>?

A

Blue berry muffin baby= toxoplasmosis

80
Q

What are the early and late signs of congenital syphilis

A

Early: maculopapular rash and snuffles

Late: Frontal bossing, Saddle nose and short maxilla

HITCHINSON TEETH, SABER SHINS, and DEAFNESS

81
Q

A pregnant pt with symmetric arthritis in the hands, feet, knees, and feet

May indicate what TORCH infection

A

Parvovirus B19

Also look for a low retic count to DDX from arthritis

82
Q

What is the causative agent of Listeria

A

Unpasteurized Dairy or Deli meats

83
Q

What is the presentation or Rubella

A

Maculopapular Rash that spreads from the head and then distally

Post auricular Lymphadenopathy

And More than 5 joint poly Arthiritis

84
Q

What is the triad of congenital rubella syndrome

A

Deafness
Cataracts
And Heart defects ( PDA )

85
Q

What threeTORCH infections present with a blueberry like rash?

A

Toxoplasmosis and Rubella and CMV

86
Q

How does CMV show up in infants MRIs?

A

Periventricular Calcification

87
Q

A neonate with polyhydroamnios think

A

Possible Bowel obstruction
(Stable course)

88
Q

What is a major differnce in gestational time line of symmetric vs Asymmetric SGA

A

Symmetric typically occurs early in the gestational course

Where as Asymmetric occurs late
Is a more concerning finding and has more risks (hypoglycemia ect.

89
Q

What are the risk factors that contribute to LGA in a newborn

A

Maternal DM

90
Q

What is the most common cause of jaundice in a newborn

A

Physiologic Hyerbillirubinemia

91
Q

Hair collar sign around a lesion in a newborn may indicate what?

A

Cutaneous signs of cranial dysraphism

92
Q

What is the next step if you find a cephalocele or a exophytic nodule on the scalp of a newborn

A

MRI

93
Q

How do you secure a newborns arm with a suspected clavicle fx

A

arm abducted more than 60 degrees and the elbow flexed more than 90 degrees.3

94
Q

What congenital mutuation is associated with a cleft palate

A

trisomy 13

95
Q

What is CHARGE association/ D/o

A

A syndrome of
-Coloboma
-heart defects
-Atresia of the Choanea
-retardation
-genital defects
-ear anomalies

Finding of any one of these should prompt evaluation for the others

96
Q

What are the signs of congential gluacoma

A

signs of glaucoma develop during the first several weeks or months of life and include corneal cloudiness and enlargement, tearing, bleph- arospasm, and photophobia.

97
Q

What are the two ABX for Gonorrhea Conjunctivitis

A

Ceftriaxone 25-50 mg/kg x1
Cefotaxime 100mg/kg x1

98
Q

What is polands syndrome

A

Unilateral absence or hypoplasia of the pectoralis major muscle suggests the diagnosis of Poland’s syndrome

99
Q

What does a bell shaped thorax in a newborn signal

A

A bell-shaped thorax is often present in newborns with neurologic abnormalities or some dwarfing syndromes.

100
Q

NML HR in newborns

A

120-160

101
Q

Scaphoid abdomen think …

A

A scaphoid abdomen suggests the presence of a diaphragmatic hernia.

102
Q

What are the nerve roots affected by a brachial plexus injury

A

C5-T3

103
Q

If an Bartlow Ortlani test is inconclusive, when should the next test be performed

A

At 2 weeks

If positive, refer to Ortho for Hip Dysplasia

104
Q

A C curve in the lateral border of a new borns foot
Is …

A

metatarsus adductus

This defect is commonly associated with a fixed intrauterine position and may be associated with developmental hip dysplasia.

105
Q

What lab can you refer to ID a Neuroblastoma

A

catecholamine levels

106
Q

What two systems must be investigated in a newborn with ambiguous genitalia

A

The presence of ambiguous genitalia is a medical emergency. Adrenal and pituitary integrity must be established.

107
Q

What are the Defib Doses in infant resus

A

2J kg-> 4J/kg then increasing to a Max of 10J/Kg

108
Q

Cardio version dose in infants

A

0.5-1 J/kg up to 2J/kg in ineffective

109
Q

What are the LEAN (V)drugs than can be given through an ET tube?

A

Lidocaine
Epi
Atropine
Naloxone
Vasopressin

110
Q

What are the 1st 6 things that are assessed in resusc of an infant

A

Pulses
Perfusion (cap refil)
Rate
rhythm
BP
UOP

111
Q

What is the Bullous infusion rate for neonate resus

A

60ml/kg in 20ml/kg increments
Even if BP in normal

112
Q

What is the IVF fluid rate in cardiac resus in a newborn/ peds

A

5-10ml/kg over 10-20 minutes

113
Q

Head bobbing in an infant may be a sign of …

A

Resp distress

114
Q

What is the flow rate for HFNC in peds pts

A

2L/kg up to 12L/kg in infants

30L/kg in adolescents / peds

Up to 50L/kg in adults

115
Q

Most common age for epiglottitis

A

Between 1 and 7

116
Q

What ABX should be initiated for a pt with Epiglottitis

A

Ceftriaxone and Vanc

117
Q

Common age group for croup

A

6-36 months

118
Q

What is the dextrose dose in a hypoglycemic pt

A

5-10ml/kg of a 10%dextrose solution

Or 2-4 ml.kg of a 25% dextrose solution

PHASE II (3 decks)

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