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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
In pts with Trisomy 21 will the PAPP-A be high or low
Low with fetal Down syndrome
26
Increased thickness of Nuchal translucency U/S can be see in what genetic abnormalities
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
27
What is the genetic disorder associated with skin hyperexentsibility, joint hyper mobility, and fragile connective tissues?
EDS ( Ehlers Danlos)
28
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?
Ehlers Danlos Meteniers Sign: easy eversion of the upper eyelid)
29
What is the mutation that causes Marfan Syndrome
Mutation of the fibrillin-1 gene resulting in weakened connective tissue
30
What is the autosomal dominant systemic connective tissue disorder that leads to Cardio, ocular, and MSK abnormalities
Marfans
31
How does Marfans present
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
32
What are the clinical manifestations of Fetal Alcohol Syndrome
Microcephaly, SMALL upper lip, smooth Philtrum, small palpebral fissures, and small distal phalanges.
33
What are the effects of smoking during pregnancy
Pre term birth, miscarriage, still birth, low Birth wt, heart defects, cleft lip/palate, malformations
34
What are the two most common neural tube defects
Spina Bifida and Anencephaly
35
What is the most common deficiency that leads to neural tube defects?
Folate
36
What is a failure of closure of the portion of the nueral tube that becomes the cerebrum
Anencephaly
37
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.
Spina Bifida
38
How do we screen for Neural Tube defects
Increased material serum Alpha Fetoprotein followed by amino centesis showing increased alpha-fetoprotein and increased acetylcholinesterase
39
What is the genetic disorder 2/2 a small deletion or inexpressioin of genes in the paternal copy of chromosome 15
Prader- Willi Syndrome
40
Genetic Disorder that presents as prenatal hypotonia, post natal growth delay, hypogonadotrphic hypogonadism, and obesity after infancy
Prader Willi (Chromosome 15) syndrome
41
A weak floppy baby, with cryptorchidism think
Prader willi syndrome
42
What is the presentation for Beckwith-Wiedmann Syndrome
Larger for gestational age, organomegaly, macroglossia, hypo-gl, asymmetric limbs
43
An infant with beckwith-Wiedmann syndrome is at an increased risk of what two neoplasms.
Hepatoblasotma and Wilms Tumor
44
A peds pt presents with ataxia, opsoclonus myoclonus syndrome, HTN, and diarrhea
Neuroblastoma Most common to the adrenal medulla or para spinal region. Opsoclonus Myoclonus Syndrome: dancing eyes and dancing feet
45
What is NF1
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
46
What does NF1 look like on MRI
Unidentified bright objects on T2 scans, in the basal ganglia, brainstem, or cerebellum.
47
What is NF2
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
48
What is the mutation in NF2
Mutation of the NF2 tumor suppressor gene (normally produces protein schwannomin aka Merlin)
49
If a peds pt presents with bilateral vestibular neuromas.. think
NF2
50
What are the optic and skin manifestations of NF2
Cataracts, retinal hamartomas, Cutaneous tumors, less assoc with cafe au lait than NF1
51
What medication can be used to treat hearing loss and shrink tumors in pts with NF2
Bevacizumab
52
What is the autosomal recessive genetic d/o most common in Ashkenazi Jewish families of Eastern Europe, also seen in Cajuns on Southern Louisiana ?
Tay-Sachs Dz
53
What is the clinical manifestation of Tay Sachs in an Infant ?
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
What is the presentation of Tay-sachs in ages 2-10
Motor skill degeneration, dysphagia, ataxia and spasticity, death occurs between 5-15
55
What is the presentation of adult onset tay-sachs
Unsteady spastic gait and prgressive deterioration leading to psychosis
56
What are the physical exam findings of a PT with tay-sachs
Cherry rep spots on the macular pallor and macrocephaly
57
Are live attenuated vaccines given to immunocomp or pregnant pts
NO!
58
What is the only live vaccine that can be given to HIV pts
MMR, CD# must be above 200.
59
What allergies are contraindicated for MMR vax
Neomycin and Streptomycin
60
What vaccines can be given in pregnancy
diphtheria, tetanus, inactivated flu, HBV, rabies, menigococal
61
What is an APGAR score
Appearance Pulses Grimace Activity Respirations
62
Define prematurity
Before 37 weeks
63
Define Late prematurity
Between 34-27 weeks
64
Be fine moderate prematurity
Between 32-34 weeks
65
Define very/ extreme prematurity
Very= before 32 weeks Extreme= before 28 weeks
66
Define LBW
Less than 2500g
67
Define intraventricular hemorrhage on the newborn
Bleeding in the germinal matrix (between the caudate nucleus) w/in the 1st day of life
68
A newborn on the 1st day of life presents with a bulging fontanelle, SZR, hypotonia, coma, and HOTN Think
Intraventricualr Hemorrhage Dx with Cranial U/S -blood in the lateral ventricles -hydrocephalus Should be screened in all pts under 30wks
69
What pts need screening for retinopathy of prematurity
All infants under 1500g or less than 30 wks
70
How do you treat apnea of prematurity (>85% o2 and >80bpm x 20secs)
Drying the baby Rubin the back/Soles of Feet Radiant warmer/ Incubator Suctioning PRN Adjust the airway BVM PRN
71
When and for what condition would you give caffine citriate to a newborn
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
What is a condition in neonates that causes a decrease in the # of alveoli, interstitial thickening, Pulm edema, and atelectasis
Broncho-pulmonary Dysplasia 2/2 Prolonged mechanical ventilation
73
How do we treat Broncho-pulm dysplasia
Supportive tx and exploration of resp infections Fluid restriction and diuretics
74
What are the TORCH infections
Toxoplasmosis Other- Rubella CMV HSV-2
75
What are the “other” infections of TORCH
Listeria Parvovirus B19 Syphillis Varicella
76
How are TORCH infections transmitted
Vertically transmitted
77
When is the infant at Risk of COngential toxoplasmosis
the 1st 2 trimesters
78
A new born presents with chorioretnitis, hydrocephalus, and intracranial calcifications What is the TORCH infection suspected?
Toxoplasmosis
79
A baby with blue-ish marks on the skin What TORCH infection>?
Blue berry muffin baby= toxoplasmosis
80
What are the early and late signs of congenital syphilis
Early: maculopapular rash and snuffles Late: Frontal bossing, Saddle nose and short maxilla HITCHINSON TEETH, SABER SHINS, and DEAFNESS
81
A pregnant pt with symmetric arthritis in the hands, feet, knees, and feet May indicate what TORCH infection
Parvovirus B19 Also look for a low retic count to DDX from arthritis
82
What is the causative agent of Listeria
Unpasteurized Dairy or Deli meats
83
What is the presentation or Rubella
Maculopapular Rash that spreads from the head and then distally Post auricular Lymphadenopathy And More than 5 joint poly Arthiritis
84
What is the triad of congenital rubella syndrome
Deafness Cataracts And Heart defects ( PDA )
85
What threeTORCH infections present with a blueberry like rash?
Toxoplasmosis and Rubella and CMV
86
How does CMV show up in infants MRIs?
Periventricular Calcification
87
A neonate with polyhydroamnios think
Possible Bowel obstruction (Stable course)
88
What is a major differnce in gestational time line of symmetric vs Asymmetric SGA
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
What are the risk factors that contribute to LGA in a newborn
Maternal DM
90
What is the most common cause of jaundice in a newborn
Physiologic Hyerbillirubinemia
91
Hair collar sign around a lesion in a newborn may indicate what?
Cutaneous signs of cranial dysraphism
92
What is the next step if you find a cephalocele or a exophytic nodule on the scalp of a newborn
MRI
93
How do you secure a newborns arm with a suspected clavicle fx
arm abducted more than 60 degrees and the elbow flexed more than 90 degrees.3
94
What congenital mutuation is associated with a cleft palate
trisomy 13
95
What is CHARGE association/ D/o
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
What are the signs of congential gluacoma
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
What are the two ABX for Gonorrhea Conjunctivitis
Ceftriaxone 25-50 mg/kg x1 Cefotaxime 100mg/kg x1
98
What is polands syndrome
Unilateral absence or hypoplasia of the pectoralis major muscle suggests the diagnosis of Poland’s syndrome
99
What does a bell shaped thorax in a newborn signal
A bell-shaped thorax is often present in newborns with neurologic abnormalities or some dwarfing syndromes.
100
NML HR in newborns
120-160
101
Scaphoid abdomen think …
A scaphoid abdomen suggests the presence of a diaphragmatic hernia.
102
What are the nerve roots affected by a brachial plexus injury
C5-T3
103
If an Bartlow Ortlani test is inconclusive, when should the next test be performed
At 2 weeks If positive, refer to Ortho for Hip Dysplasia
104
A C curve in the lateral border of a new borns foot Is …
metatarsus adductus This defect is commonly associated with a fixed intrauterine position and may be associated with developmental hip dysplasia.
105
What lab can you refer to ID a Neuroblastoma
catecholamine levels
106
What two systems must be investigated in a newborn with ambiguous genitalia
The presence of ambiguous genitalia is a medical emergency. Adrenal and pituitary integrity must be established.
107
What are the Defib Doses in infant resus
2J kg-> 4J/kg then increasing to a Max of 10J/Kg
108
Cardio version dose in infants
0.5-1 J/kg up to 2J/kg in ineffective
109
What are the LEAN (V)drugs than can be given through an ET tube?
Lidocaine Epi Atropine Naloxone Vasopressin
110
What are the 1st 6 things that are assessed in resusc of an infant
Pulses Perfusion (cap refil) Rate rhythm BP UOP
111
What is the Bullous infusion rate for neonate resus
60ml/kg in 20ml/kg increments Even if BP in normal
112
What is the IVF fluid rate in cardiac resus in a newborn/ peds
5-10ml/kg over 10-20 minutes
113
Head bobbing in an infant may be a sign of …
Resp distress
114
What is the flow rate for HFNC in peds pts
2L/kg up to 12L/kg in infants 30L/kg in adolescents / peds Up to 50L/kg in adults
115
Most common age for epiglottitis
Between 1 and 7
116
What ABX should be initiated for a pt with Epiglottitis
Ceftriaxone and Vanc
117
Common age group for croup
6-36 months
118
What is the dextrose dose in a hypoglycemic pt
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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