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Uworld 13 Flashcards

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1
Q

Management of traumatic brain injury (concussion)

A

head CT scan w/o contrast

observation for 4-6 hours

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2
Q

at what age can a child state their age and their gender

A

3

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3
Q

what age can a child understand the irreversibility of death?

A

7-8

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4
Q

rapid hematoma expansion in an epidural hematoma can turn into

A 
elevated intracranial pressure (impaired consciousness
uncal herniation (ipsilateral oculomotor nerve palsy)
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5
Q

manangement of febrile seizure

A

most are discharged home with no evaluation

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6
Q

fetal alcohol syndrome facial features

A

palpebral fissues
smooth philtrum
thin vermilion borders
microcephaly

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7
Q

Fragile X syndrome: facial features

A

prominent jaw
large ears
macroorchidism

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8
Q

how is Friedriech ataxia inherited

A

autosomal recessive

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9
Q

Parents have one child who is has friedriech ataxia, she is pregnant and worried about her 2nd child have the disability. What do you advice her?

A

genetic counseling

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10
Q

newborn or young infant with failure to thrive, bilateral cataracts, jaundice and hypoglycemia

A

galactosemia

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11
Q

what is the enzyme deficiency for galactosemia

A

galactose-1-phosphate uridyl transferase deficiency

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12
Q

a newborn or young infant with only cataracts and otherwise asymptomatic? enzyme deficiency

A 
Galactokinase deficiency 
(Uridyl diphosphate galactose-4-epimerase deficiency)
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13
Q

difference between homocytinuria and Marfan

A

Homocytinuria:
intellectual disability
downward lens dislocation
hyper coagulability

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14
Q

how do you treat homocystinuria

A

vitamin supplementation

antiplatelet or anticoagulation to prevent thromboembolic events

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15
Q

next step in management for a child who comes in with a big head circumference and signs of increased intracranial pressure

A

CT scan of brain

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16
Q

inability to extend the knee while the hip is flexed?

A

Kernig sign = meningitis

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17
Q

Most common cause of acute and recurrent headaches in pediatric population

A

migraine

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18
Q

treatment for migraine

A

1st: NSAIDs
2nd: Triptans

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19
Q

prophylactic treatment for migraines

A

topiramate
beta blockers
amitriptyline

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20
Q

how is myotonic dystrophy inherited

A

autosomal dominant

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21
Q

what are some distinctive clinical features of myotonic dystrohpy

A

facial weakness
hand grip
cataracts

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22
Q

what is seen in x-ray and CT scan for neuroblastoma? and lab?

A

calcifications and hemorrhages

serum and urine catecholamines and HVA and VMA elevated

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23
Q

a child has optic glioma. what do you associate that with?

A

Neurofibromatosis type 1

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24
Q

Brushfield spots in eye

A

Down Syndrome

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25
what is the clinical difference between Niemann-Pick disease vs. Tay-Sachs disease?
Nieman pick: hepatosplenamegaly and areflexia | Tay-Sachs: hyperreflexia
26
Intellectual disability fair complexion eczema musty or "mousy" body order
PKU | Phenylketonuria
27
Adlose B deficiency
Hereditary fructose intolerance
28
absent galactose-1-phosphast uridyl transferase activity
galactosemia
29
Lennox-gastaut syndrome
intellectual disability | severe seizures of varying types
30
3-Hz spike-wave discharges during episodes
absence seizure
31
``` Port wine stain leptomeningeal capillary-venous malformation seizures glaucoma intellectual disability ```
Struge-Weber
32
Diagnostic study of Struge-Weber
MRI of brain with contrast
33
PANDAS
pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections
34
frist line treatment for Tourette
clinidine
35
A child with meningococcemia with sudden vasomotor collapse and skin rash due to adrenal hemorrhage?
Waterhouse-Friderchsen
36
what bacteria do you think of for dog or cat bite?
pasteurella multocida
37
is fever and mild rash after MMR vaccine okay
yes!
38
clinical feature of scarlet fever
fever pharyngitis sandpaper, maculpapular rash
39
1-mm gray vesicles on the tonsillar pillars and posterior oropharynx that progress to fibrin-coated ulcerations
herpangina--> coxsackie A virus --> supportive management
40
when do you give varicella immunization to children
1 and 4
41
rules for varicella post-exposure prophylaxis
got vaccines: observe no, immunocompetent: give vaccine no, immunocompromised: VZIG
42
Who is not eligible for either varicella vaccine immunoglobulins
children under 1 age
43
Enterobius vermicularis? treatment?
pinworm | albendazole or pyrantel pamoate
44
treatment for Strongyloidiasis
Ivermectin
45
Treatment for acute airway obstruction from EBV
corticosteroids
46
Chronic inspiratory stridor that begins in the neonatal period and is worse in the supine position.
Laryngomalacia: collapse of supraglottic structures | so infectious symptoms
47
most common cause of acute, unilateral cervical lymphadenitis in children
Staph aureus
48
most common causes of meningitis in children less than 3 months
Group B strep E. coli Listeria HSV
49
most common causes of meningitis in children 3 months to 10 years
Streptococcus Pneumoniae | Neisseria meningitidis
50
most common causes of meningitis in children greater than 11
Neisseria meningititids
51
treatment for Haemophilus influenza type B meningitis
Dexamethasone
52
most common cause of viral meningitis in children
echovirus | coxackie virus
53
acute poor feeding, jaundice, and lethargy. well until 2 days ago
congenital hypothyroidism
54
most common cause of osteomyelitis in healthy children
Staphylococcus aureus
55
how is the pertussis diagnosis confirmed
PCR testing of nasopharynx
56
center criteria. meaning
fever tender anterior cervical lymphadenopathy tonsillar exudates absence of cough 4: abs 2-3: test 0-1: not it
57
maternal-fetal transmission of rubella virus is most teratogenic during what trimester
first
58
what viruses make a mother have a C-section
HIV and HSV
59
describe the sandpaper rash in Scarlet fever
prominent along skin folds(axillae, groin) | desquamation ( as illness resolves, desquamation of the rash results in peeling of hands and feet).
60
most common cause of osteomyelitis in sickle cell patients
salmonella | Staph. aureus
61
how is sickle cell inherited
autosomal recessive
62
Maintenance for a child who was born with sickle cell anemia
pneumococcal vaccine penicillin until age 5 Folic acid supplementation hydroxyurea
63
most common cause of acute bacterial rhinosinusittis
1. Strep pneumo | 2. Monty-able H. flu
64
most common cause of chronic bacterial rhinosinusitits
inflammation of rhino sinuses for greater than 12 weeks | staph. aureus
65
treatment for impetigo skin
mupirocin
66
specific findings that is highly suggestive of congenital syphilis are
copious rhinorrhea | maculopapular rash that may desquamate or become bullous
67
what causes umbilical stump infection? clinical feature?
neonatal tetanus - trismus - spasms - hypertonicity
68
main difference between botulinum and tetanus
botulinum: hypotonic tetanus: hypertonic
69
what is a risk factor for toxo you don't thin of
unwashed fruits/vegetables
70
what can be done in a child when an emergency access is necessary and peripheral access cannot be obtained
intraosseous vascular access
71
General rule for wet diapers
number of wet diapers = age in days for first week | after first week = greater than 6 diapers
72
when is birth weight regained
age 10-14
73
What are "pink stains" or "brick dust" in neonatal diapers
uric acid crystal normal no treatment
74
what sexual development disorder has minimal to absent axillary/pubic hair
complete androgen insensitivity syndrome
75
What is the phenotype and genotype for complete androgen insensitivity syndrome
phenotype: female genotype: 46XY
76
what is management of complete androgen insensitivity syndrome
bilateral gonadectomy after completion of puberty | -decreases risk of gonadal malignancy
77
3 things you first no matter what in primary amenorrhea
1. FSH, if no breast development 2. If FSH, decreased. Pituitary MRI 3. FSH increased, Karyotyping
78
First step in management of Turner syndrome
pelvis U/S to evaluate internal female anatomy
79
Immunostaining for terminal deoxynucleotideyltransferase (TdT)
ALL
80
Diamond-Blackfan anemia
- presents in first 3 months of life with pallor and poor feeding - PURE RED CELL APHASIA - cleft palate, webbed neck and triphalangeal thumbs
81
treatment for Diamond-Blackfan anemia
corticosteroids | red blood cell transfusions
82
Fanconi anemia
pancytopenia bone marrow failure | congenital anomales
83
most common primary bone tumor in children and young adults and typically involves the metaphyses of long bones
osteosarcoma
84
Limited upward gaze bilateral eyelid retraction light-near dissociation
parinaud syndrome | pineal gland masses
85
"dancing eyes and feet"
neuroblastoma
86
average age for Fanconi's anemia
8 years
87
hemosiderin deposition leading to synovitis and fibrosis within the joint. who gets it?
Hemophilia
88
difference between hemarthrosis and hemosiderin
hemarthrosis: acute onset hemosiderin: more chronic
89
what is considered excessive intake of cow's milk
greater than 24 oz
90
difference between iron deficiency thalassemia and iron deficiency
elevated red cell distribution in iron deficiency anemia
91
children who develop isolated thrombocytopenia and petechiae after viral infection
immune thrombocytopenia
92
treatment for immune thrombocytopenia
skin only: observation | bleeding: IVIG or steroids
93
what prophylaxis is needed for sickle cell anemia
pneumococcal vaccination plus penicillin prophylaxis
94
define polycythemia in neonate
hematocrit greater than 65 percent
95
what are 3 risk factor for neonatal polycythemia vera
delayed cord clamping maternal hypertension maternal diabetes mellitus
96
what is the primary mechanism of action of hydroxyurea
increase fetal hemoglobin | which dilutes the amount of sickle hemoglobin
97
Difference between aplastic crisis and splenic sequestration crisis in acute severe anemia in sickle cell disease
AC: decrease reticulocyte count SSC: increased
98
most common complication of sickle cell
painless hematuria
99
what is physiologic jaundice
unconjugated hyperbilirubinemia | after frist 24 hours of life, resolves within a week of life
100
what diagnosis muscular duchenne and what is gold standard confirmation
diagnosis: muscle biopsy | gold standard confirmation: genetic testing

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