Peds Uworld Flashcards
(131 cards)
1
Q
cyanosis and single S2 (most common cardiac defect of neonatal period)
in contrast, what is the most common after the neonatal period?
in contrast, what will present with shock due to impaired systemic perfusion?
A
transposition of the great vessels
after: ToF
shock: coarctation of aorta and hypoplastic left heart
2
Q
recurrent skin/mucosal infections, periodontal disease, ^WBCs with neutrophil predominance, think?
A
Leukocyte adhesion deficiency
also: lack of umbilical cord separation and no pus
3
Q
acute edema of face, extremities, genitals, trachea, abdominal organs without urticaria, think?
A
hereditary angioedemia
C1 inhibitor deficiency
leads to ^bradykinin and C2b (edema-producing factors)
most common cause of acquired form is from using ACE-i
4
Q
recurrent infections with S. aureus, Serratia, Burkholderia, Aspergillus, think ? what test to get?
A
Chronic Granulomatous Disease: impaired oxidative burst, NADPH oxidase deficiency, get inf. with catalase+ organisms
think Cats Need PLACESS to Belch their Hairballs
Nocardia, Pseudomonas, Aspergillus, Candida, E. coli, Staph, Serratia, Burkholderia, H. pylori
get dihydrorhodamine 123 test (old: nitroblue)
5
Q
partial albinism and recurrent cutaneous infections with S. aureus and S. pyogenes, think?
A
Chediak-Higashi
6
Q
eczema, thrombocytopenia, recurrent infections, think?
A
Wiskott-Aldrich
impaired cytoskeleton changes in WBCs/plts
tx: SCT
7
Q
fever, lethargy and signs of heart failure after a viral prodrome, think?
how to manage?
A
viral myocarditis
ICU due to risk of acute decompensation and fatal arrhythmia
+/- cardiomegaly and pulmonary edema on CXR
8
Q
failure to thrive, recurrent infections of all types, low lymphocyte and T cell concentrations, think?
tx?
A
SCID
tx: stem cell transplant
9
Q
recent Giardia infection and lobar pneumonia, consider ?
A
Bruton’s X-linked agammaglobulinemia: abnormal B lymphocyte maturation, at risk for sinopulmonary infections with encapsulated orgs (H. flu, S. pyogenes) due to imp. humoral response, and GI infections i.e. Giardia due to absence of IgA
10
Q
contraindications to DTaP
A
anaphylaxis to vaccine ingredients
additional for pertussis part: progressive neurological disorder or encephalopathy within wk of vaccine
11
Q
tx for long QT syndrome
A
B-blockers (propranolol) with pacemakers (except sotalol which blocks K+ channels)
12
Q
electrolyte derangements that can cause prolonged QT
A
low Ca2+, low K+, low Mg2+
13
Q
meds that can cause prolonged QT
A
macrolides, FQs, antipsychs, TCAs, SSRIs, methadone, oxy, zofran, quinidine, procainanmide, flecainide, amiodarone, sotalol
14
Q
failure to thrive and recurrent respiratory and GI infections, chronic lung disease especially with Giardia, encapsulated bacteria, and enterovirus, think?
A
common variable immunodeficiency: B-cell differentiation impaired leading to decreased of all Igs
15
Q
wheezing, coughing, dysphagia, biphasic stridor that improves with neck extension, think?
what improves with prone positioning?
A
vascular rings
prone: laryngomalacia (collapse of supraglottic structures during inspiration)
16
Q
should you tx rheumatic fever with abx?
A
yes, PCN, as they are at risk of recurrent episodes and progression of rheumatic HD with repeated infection with GABS pharyngitis
17
Q
what is never normal on neonatal EKG?
A
left axis deviation
if present + decreased pulmonary markings on CXR, think tricuspid atresia (hypoplasia of RV and pulm. outflow tract)
18
Q
AOM orgs
A
S. pneumo, H. flu (not Hib), Moraxella
tx: amoxicillin, then augmentin
19
Q
how to visualize laryngomalacia
A
direct laryngoscopy
20
Q
tinea capitis tx
A
oral terbinafine, griseofulvin, itraconazole, fluconazole
21
Q
bruise looking rash on minority kiddo
A
Mongolian spots, self-resolve during childhood
22
Q
tinea corporis treatment
A
topical clotrimazole, terbinafine
2nd line: oral terbinafine, griseofulvin
23
Q
hemangiomas in kiddos
A
strawberry (superficial)
vs cherry in adults
24
Q
proptosis, ophthalmoplegia (pain with moving eyes), diplopia, think?
most common predisposing factor?
A
orbital cellulitis
bacterial sinusitis
25
neonate with blanching erythematous papules and pustules, think ?
erythema toxic neonatorum, benign and resolves within 2 weeks
26
bedwetting is normal before age ? and does not warrant treatment before this time
age 5
27
conjunctival injection, tarsal inflammation, pale follicles, think ?
trachoma: C. trachomatis A, B, C infection, leading cause of blindness worldwide
tx: oral azithromycin, may need eyelid sx
28
macrosomia, hypoglycemia, macroglossia, umbilical hernia/omphalocele, hemihyperplasia (1 side bigger than other) think ?
what to do next?
Beckwith-Wiedemann syndrome
get abdominal U/S to check for development of Wilms tumor or hepatoblastoma
in contrast, hypothyroidism may have macroglossia and umbilical hernia but not the other features
29
conjugated hyperbili + hepatomegaly in a newborn, think?
what to do next?
treatment?
biliary atresia
dx: abdominal U/S
tx: Kasai procedure
30
Reye syndrome:
presentation
labs
biopsy
n/v, encephalopathy, acute liver failure
^LFTs, ^PT, INR, PTT, ^NH3
microvesicular steatosis
in contrast: macrovesicular fatty changes are seen in alcoholic hepatitis and in NAFLD
31
1 month old with painless blood-streaked stools, eczema, regurgitating feedings, think?
milk- or soy-protein proctocolitis (enterocolitis/allergy)
32
intussusception dx? tx?
dx: U/S: "target sign"
tx: air or water-soluble contrast enema
33
see microcolon on contrast enema, think?
```
meconium ileus (at the level of the ileum), will also have thick "inspissated" meconium
in contrast, Hirschsprung will have a "transition point" on contrast enema (typically rectosigmoid)
```
34
infant supplement timeline
all exclusively breastfed infants: 400 IU vitamin D 1st mo
breastfed + preterm infants: iron at birth-1 yr
can introduce pureed foods at 6 mos
can introduce cow's milk at 1 yr
35
vitamin B6 (pyridoxine) deficiency presents how?
cheilosis, stomatitis, glossitis + irritability, confusion, depression
in contrast, riboflavin (B2) def. presents with cheilosis, stomatitis, glossitis + normocytic anemia, seborrheic dermatitis
36
15 day old with bilious vomiting and no gas seen on abdominal XR, think ? what to do next?
treatment?
malrotation with volvulus (both meconium ileus and Hirschsprung will have some gas, those are dx with contrast enema)
dx: upper GI series (barium swallow), will see Ligament of Treitz on right side of abdomen (malrotation), and "corkscrew" pattern (volvulus)
tx: Ladd procedure (fix bowel)
37
why do newborns get jaundiced?
| at what levels to do exchange transfusion?
^hgb turnover/bili production
decreased UGT activity (conjugating enzyme)
sterile gut cannot break down bili to urobilinogen
if total bilirubin exceeds 20-25 mg/dL, otherwise can use phototherapy
38
where it is worrisome for batteries to get lodged
esophagus: needs immediate endoscopic removal to prevent mucosal damage and esophageal ulceration
if distal, 90% pass uneventfully
39
8 yo: RUQ pain, dark urine, jaundiced, ^bili (D, T), ^amylase, ^lipase, +extrahepatic cyst on u/s, think?
biliary cyst: cause cholestasis, palpable mass, +/- pancreatitis
tx: surgical resection
f/u: cholangiocarcinoma
40
"lead points" in intussusception
most common: hypertrophy of Peyer patches from recent illness
others: Meckel's, HSP, celiac, tumor, polyps
other risk factors: rotavirus vaccine
41
intussusception tx
U/S guided air contrast enema or saline enema
| barium enema not done due to risk of peritonitis from barium leakage if intestine perforates
42
rehydration for dehydrated kiddos
mild-mod: oral
| mod-severe: IV NS, (dextrose-containing fluids may be used in maintenance, but not resuscitation)
43
weight loss, iron-def anemia, vesicular skin rash, T1 DM, think?
celiac
| also associated with IgA deficiency, Downs, AI thyroiditis
44
constitutional growth delay
normal birth weight/height
then drop to 5th/10th % btw 6mo-3yrs, remains low until growth spurt, should reach normal adult height
giving GH will NOT help, they are not deficient
45
3 mo old: hypoglycemic, lactic acidosis, hyperuricemia, hyperlipidemia
doll-like face, thin extremities, hepatomegaly
Glucose-6-phosphatase deficiency (type 1 glycogen storage disease, von Gierke disease)
cannot convert glycogen to glucose
in contrast, glucocerebrosidase deficiency (Gaucher) presents with bone pain, cytopenias, hepatosplenomegaly, but NOT hypoglycemia and lactic acidosis
46
Turners hormone levels
low estrogen, progesterone ("streak ovaries")
| ^FSH, LH (disinhibited feedback)
47
most common cause of congenital hypothyroidism ww
thyroid dysgenesis
| in contrast, transplacental passage of TSH-rec Abs leads to hyperthyroidism
48
when to remove testes in AIS
after puberty so chica gets to full height but reduces risk of malignancy
49
lead poisoning tx
moderate 45-69: DMSA
| severe 70+: DMSA + EDTA
50
precocious puberty with low LH levels that do not ^after GnRH stim test
nonclassic CAH (21-hydroxylase deficiency)
51
primary amenorrhea w/u
FSH if no breast development
if FSH decreased: pituitary MRI (prolactinoma)
if FSH increased: karyotype
52
abdominal pain, shock, hematemesis, metabolic acidosis + radio opaque areas on XR think ?
tx?
iron poisoning, tx with deferoxamine
53
airborne vs droplet vs contact diseases
airborne: measles
droplet: influenza, RSV
contact: MRSA, rotavirus
54
most common causes of acute, u/l LAD in kids
S. aureus, S. pyogenes
55
Lyme tx for kids <8
amoxicillin PO or cefuroxime
56
impetigo tx
topical mupirocin
57
posterior oropharyngeal vesicles/ulcerations, fever, pharyngitis, think?
herpangina (coxsackie virus)
| tx: supportive
58
all nonimmune, asymptomatic pts older than 1 yr with varicella exposure should be treated how?
post-exposure ppx with varicella vaccine
| if immunocompromised get VZIG
59
Cat-scratch vs cat bite abx
Cat-scratch: azithromycin
| Cat bite: augmentin (Pasteurella)
60
osteomyelitis tx
nafcillin/oxacillin or cefazolin
if suspect MRSA: vancomycin or clindamycin
if SCD: same as above + ceftriaxone/cefotaxime
61
most common predisposing factor for acute bacterial sinusitis
viral URI
62
prophylaxis for close contacts of pertussis?
macrolides for all, regardless of age, immunization status, or symptoms
63
most common cause of sepsis in SCD pt
S. pneumo (encapsulated, N. men and Hib are less common due to vaccinations)
Salmonella in osteomyelitis, not sepsis
64
hydroxyurea SE
myelosuppression
65
ALL markers
Tdt+, PAS+, no peroxidase + granules
66
pure red cell aplasia + webbed neck, short stature, cleft lip, shield chest, thumb anomalies, think?
diamond-blackfan anemia
67
medulloblastoma location/symptoms
posterior fossa, from cerebellar vermis
truncal/gait ataxia
obstructive hydrocephalus and s/s of ^ICP (near 4th ventricle)
68
kid with a stroke, think ? what to do next?
SCD, get Hgb electrophoresis
| most common cause ped. stroke
69
labs in splenic sequestration
low Hgb, ^retics, low plts
| in contrast, aplastic crisis will have normal plts but low retic count
70
limitation of upward gaze, think?
Parinaud syndrome, occurs with pinealoma
| in contrast, craniopharyngiomas (suprasellar) can cause visual field deficits
71
s/s of polycythemia in neonate
respiratory distress, hypoglycemia, neurological manifestations
72
craniopharyngioma vs pituitary tumor
both can cause visual field deficits and hormone derangements
craniophayngiomas have calcification and light up on CT/MRI
73
anemia of prematurity
low EPO, low Hbg, HCT, and retic count
74
poor growth, macrocytic anemia, congenital marrow failure, short stature, other physical anomalies (skin, digits), think?
```
Fanconi anemia (AR)
DNA repair defect causing chromosomal breaks
```
75
aplastic anemia vs aplastic crisis
anemia: pancytopenia due to BM failure (congenital or acquired)
crisis: just anemia, typically in SCD pt exposed to Parvovirus B19 (arrest of EPO, low retic)
76
kiddo develops isolated thrombocytopenia (low plts) and petechia after viral infection, think ? how to manage?
immune thrombocytopenia (ITP)
kids typically recover spontaneously in 6 mos, only need observation (regardless of platelet count)
BUT if bleeding should receive IVIG or steroids
in contrast, adults with plts less than 30,000 or bleeding need IVIG/steroids
77
nuclear remnants within RBCs in asplenic pts
ribosomal precipitates with thals or lead
small inclusions seen in G6PD def.
Howell-Jolly bodies
Basophilic stippling
Heinz bodies
78
iron def labs
low MCV, ^RDW, low iron, low transferrin sat, ^TIBC, low RBC count
in contrast, thalassemia has a normal RDW and RBC cell count
79
sepsis in a SCD kid, think?
pneumococcal, ppx with conjugate capsular polysaccharide vaccine and PCN
80
SCD can lead to what vitamin deficiency?
folate (^reticulocytosis)
81
what is hemophilic arthropathy seen in hemophiliacs caused by?
iron/hemosiderin deposition leading to synovitis and fibrosis in the joint
(prolonged PTT)
82
dx of ALL
+25% lymphoblasts on bone marrow biopsy
83
most common complications of bacterial meningitis
hearing loss!
| others: intellectual injury, CP, learning disabilities, seizure disorder
84
absence seizure disorder is associated with
ADHD
85
cherry red macula, hepsplenmeg, areflexia, feeding difficulties (2-6 mos), think?
sphingomyelinase deficiency: Niemann-Pick
| IN CONTRAST, Tay-Sachs has same presentation but HYPERREFLEXIA and NO hepslenmeg
86
Krabbe disease?
Gaucher?
Hurler?
Krabbe: galactocerebrosidase deficiency (hypotonia, areflexia, regression)
Gaucher: GLUCocerebrosidase deficiency (anemia, low plts, hepsplenmeg)
Hurler: lysosomal hydrolase deficiency (coarse features, hernias, corneal cloud, hepsplenmeg)
87
Marfan vs Homocystinuria
Marfan: AD, NORMAL intellect, aortic root dilation, UPWARD lens dislocation, Fibrillin-1 gene
Homo: AR, intellectual disability, +thrombosis, DOWNWARD lens dislocation, meg. anemia, fair skin
88
fever, HA, FNDs (classic triad)
| +/- seizure in kiddo with congenital HD and recurrent sinusitis, think?
brain abscess
^risk for direct spread: OM, mastoiditis, sinusitis, dental infection
^risk blood spread: cyanotic HD (bypass lung), other infected sites (seed GW-J)
89
CGG repeats, think?
Fragile X: MCC inherited intellectual disability
| prominent jaw, large ears, macroorchidism
90
stroke s/s after fall with toothbrush in mouth, think?
carotid artery dissection or thrombus formation
91
FTT, b/l cataracts, jaundice, hypoglycemia, think?
galactosemia: galactose-1-phosphate uridyl transferase deficiency
may lead to MR, cirrhosis, ^risk E. coli sepsis
tx: eliminate galactose
mild (only cataracts) if galactokinase deficiency
92
kids with Downs are at ^risk what malignancy
ALL
93
LE weakness more than UE
Werdnig Hoffman (spinal muscular atrophy)
94
infant botulism is from ?
how to tx?
in contrast to food-borne
C. botulinum spores (environment)
tx: human-derived botulism Ig
IN CONTRAST, food-borne is from ingestion of preformed toxin, tx: equine-derived antitoxin
BOTH have descending flaccid paralysis
95
LOC, post-octal state, transient hemiplegia (1-sided paralysis), think?
Todd paralysis
transient, focal weakness after focal/generalized seizure
r/o other causes with CT/MRI if no clear hx of preceding seizure
96
most common brain tumor in kids
astrocytoma
97
```
brain tumor presentations
supretentorial:
posterior fossa:
brainstem:
SC:
```
supretentorial (astrocytoma, craniopharyngioma): ^ICP, seizures, sensory changes
posterior fossa (medulloblastoma, ependymoma): ^ICP, ataxia, clumsiness
brainstem: ataxia, clumsiness, CN palsies
SC: back pain, weakness, abnormal gait
98
risk factors for IVH
premies, LBW
99
if brief loss of consciousness, HA, and vomiting after head injury, what to do?
get head CT without contrast as it is a mild TBI
100
cavernous hemangioma along trigeminal nerve distribution + intra-cranial calcifications that resemble a train-track
Sturge-Weber
101
Uncoordinated and limited voluntary movements, think? What are the most common risk factors?
Cerebral palsy
Prematurity, intrauterine growth restriction, alcohol consumption, multiple gestation, tobacco use
Comorbidities: epilepsy strabismus scoliosis
102
A two-year-old should have how many words in their vocabulary?
More than 50 and be able to combine them into two word phrases
103
Delayed muscle relaxation, facial weakness, foot drop, dysphasia, cardiac conduction anomalies, cataracts, testicular atrophy, baldness, think?
Myotonic muscular dystrophy, autosomal dominant expansion of the CTG repeat
Later onset than other dystrophies, age 12 to 30
104
Wilms tumor versus neuroblastoma origins
Wilms tumor: metanephros, precursor of the renal parenchyma
| Neuroblastoma: neural crest cells, precursor of the sympathetic chains and adrenal medulla
105
Wide-based, unsteady gait, weakness and lower limbs, decreased vibratory in position sense in the extremities, atrophy of the cervical spine accord, T-wave inversion's on EKG, think?
Friedreich ataxia, autosomal recessive, GAA nucleotide repeats, leads to myocarditis and cardio myopathy, arrhythmias, CHF
Genetic counseling is recommended
106
Amenorrhea, short stature, anosmia, delayed development, low FSH and LH, normal uterus and ovaries, think?
Kallmann syndrome
| 46, XX
107
Tourette's is associated with increased risk of?
| Tourette's treatment?
ADHD and OCD
| Treatment: antipsychotics: Risperidone, alpha 2 adrenergic receptor agonist: clonidine, Guanfacine
108
Gold standard for muscular dystrophy diagnosis
Genetic studies
| Supportive diagnostic tests: elevated CK, aldolase; fibrosis and fatty infiltration on calf muscle biopsy
109
Precocious puberty, pigmentation, polyostotic fibrous dysplasia think?
McCune Albright syndrome
| Pigmentation: café au lait spots
110
Edema in Turner's syndrome is from?
Abnormal development of the lymphatic system
111
Hypotonia, hyperphagia, obesity, think? What genetic abnormality?
Prader Willi syndrome, loss of paternal copy of 15q11-q13
Loss of maternal copy results in Angelman syndrome, (paternal uni parental disomy): short stature, intellectual disability, abnormal smiling/laughter, hand flapping, ataxia, seizures
112
Ataxia, scoliosis, cardio myopathy, Think? Most common cause of death?
Friedrich ataxia, most common cause of death is cardiomyopathy or respiratory complications
113
Risk factors for RDS
Prematurity, maternal diabetes, C-section
| Hi levels of insulin antagonize cortisol and block surfactant production
114
Diagnosis of minimal change disease
Not required, highly responsive to steroids
115
Toddler with the firm, smooth, unilateral abdominal mass and hemateria, think?
Wilms tumor, the most common pediatric renal malignancy, peaks age 2 to 5, Does not cross midline
In contrast, neuroblastoma happens more commonly in the first year of life, affects the adrenal gland, crosses the midline and has systemic symptoms
116
African-American boy with asymptomatic hemateria think?
Renal papillary necrosis due to Sickle cell trait
117
If subcutaneous emphysema, get? To rule out?
Get chest x-ray to rule out pneumothorax
| May occur secondary to severe coughing
118
2 to 24 month old with febrile UTI, get? To evaluate?
| If recurrent UTIs also get? To evaluate for?
Get Renal ultrasound to evaluate for anatomic abnormalities
| If recurrent, get a VCUG to evaluate for vesicoureteral reflux
119
First line medical treatment for enuresis if behavioral an alarm therapy fails
Desmopressin
| TCAs: imipramine, are just as effective but have more serious side effects
120
First steps If newborn with respiratory compromise and suspected diaphragmatic hernia
Endotracheal intubation, gastric tube to decompress the stomach and bowel
Bag mask ventilation can exacerbate respiratory decline
Get CXR when stable
121
Nephrotic syndrome in kid with hepatitis B think?
Membranous nephropathy
122
Complications of bronchiolitis
Apnea and respiratory failure
123
CF kid with pneumonia, treated with Tamiflu, cefepime and ? for ?
Vancomycin for S. aureus, most common cause of bacterial pneumonia in young kids with cystic fibrosis especially with coexisting influenza
In contrast, Cipro would be appropriate for adults with CF to treat Pseudomonas
124
Upper motor neuron findings in a kid with down syndrome, think?
Atlantoaxial instability
125
Kawasaki disease is a vasculitis characterized by fever for at least five days and 4/5 of the following:
Conjunctivitis, extremity changes, cervical LAD, oral mucosal changes (strawberry tongue), rash
126
Migratory Polyarthritis, pink rash with sharp edges,fever, elevated ESR, think?
Acute rheumatic fever
127
Transient Cinnabay this versus Legg-Calve-Perthes disease
Transient synovitis should self resolve in 1 to 4 weeks
128
Solitary, painful,lytic bone lesion plus Hypercalcemia in a kid, think?
Think neoplastic process: Langerhans cell histiocytosis
| In contrast, hyperparathyroidism typically occurs in patients over 50
129
Fever, rash, joint pain 1 to 2 weeks after exposure to penicillins or sulfa drugs, think?
Serum sickness like reaction
Type 3 hypersensitivity
Treatment: remove offending agent, supportive care, steroids if severe
In contrast, penicillin in the setting of EBV causes a rash that spares the extremities and does not have joint pain
130
think compartment syndrome if?
Six P's:
| Pain, pallor, poikilothermia, paresthesias, and late findings of pulselessness, paralysis
131
Isolated polyarthralgia in a kid, think?
| Lab values?
Juvenile idiopathic arthritis
| Elevated ESR, CRP, elevated ferritin, elevated IgG, elevated platelets, anemia
