Pediatrics Flashcards

1
Q

kid with macrocytic anemia, low retic count and congenital anomalies, suspect what dX?

A

Diamond-Blackfan syndrome aka congenital hypoplastic anemia

see elevated fetal hemoglobin levels

tx: corticosteroids or transfusion therapy

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2
Q

adolescent with nasal obstruction, visible nasal mass and frequent nosebleeds

A

juvenile angiofibroma

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3
Q

4 yo with hypoglycemia, hyperuricemia, hyperlipidemia and lactic acidosis, doll-like face

A

Von Gierke’s

Type 1 glycogen storage disorder caused by deficient glucose-6-phosphatase

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4
Q

patients with precocious puberty and high LH, next step?

A

brain MRI

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5
Q

what immune deficiency predisposes to Giardia infections

A

IgA deficiency

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6
Q

kid is peeing protein, best first test?

A

repeat test in 2 weeks, then quantify with 24 hour urine

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7
Q

Tx for erythema toxicum?

A

reassurance

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8
Q

> 25% lymphoblasts is diagnostic for what illness

A

ALL

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9
Q

chorioretinitis, hydrocephalus and intracranial calcs

A

toxo

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10
Q

mgmt of kid with epiglottitis

A

endotrach intubation in the OR

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11
Q

mgmt of kid with bilious vomiting and decreased stool output

A

gastrograffin enema

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12
Q

newborn with cyanosis that is aggravated by feeding and resolves with crying

A

choanal atresia

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13
Q

MC complication of sickle cell trait

A

painless hematuria

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14
Q

mgmt newborn with scaphoid abdomen and respiratory distress

A

congenital diaphragmatic hernia

endotrach intubation

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15
Q

mgmt aplastic crisis in sickle cell

A

transfusion

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16
Q

when consider formula supplementation in a neonate

A

if loses >7% of birth weight

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17
Q

15yo with progressive muscle weakness, atrophy of thenar and hypothenar eminences, testicular atrophy? Dx? inheritance?

A

Myotonic dystrophy

AD

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18
Q

when intervene in a kid with cryptorchidism?

A

if testes haven’t dropped within 6 months of birth

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19
Q

newborn with irritability, high pitched cry, diaphoresis, sneezing: what drug did mom abuse?

A

heroin

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20
Q

kid with white eye reflex, next best step?

A

refer to optho

retinoblastoma until proven otherwise

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21
Q

pt with croup with respiratory failure, next step?

A

Rac Epi before intubate, decreases need for intubation

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22
Q

child with acute rheumatic fever with valvular dz

A

all pts wtih rheumatic fever should be treated with PCN. duration depends, but at least til 21 or in some cases 40 yo

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23
Q

girl with precocious puberty, pigmentation adn polostotic fibrous dysplasia, dx?

A

McCune Albright

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24
Q

Hep B is associated with what kind of nephropathy?

A

membranous!

see low C3

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25
mgmt of pubertal gynecomastia
no workup or tx usually self resolves within 2 years
26
when begin visual acuity testing?
3 years old
27
in premature babies, what increases the risk of iron deficiency anemia as infants?
introduction of cows milk before age 12 months
28
soft tissue swelling in feet and hands of baby with sickle cell
dactylitis
29
Cri du chat gene is on what chromosome?
5p
30
tympanic membrane with peripheral granulation and some skin debris--dx?
Choelsteatoma 2/2 chronic middle ear dz retraction pocket in the tympanic membrane
31
kid presents with toxicity with anticholinergic sx | what med? what tx?
TCAs | Tx: sodium bicarb if wide QRS >100
32
RF for intussuception in older kids
Meckel's diverticulum
33
kid exposed to chicken pox, nml immunity, no h/o vaccination, next step
vaccinate! | only give IVIG if immunocompromised
34
triple bubble sign and gasless colon: dx? Rf?
jejunal atresia | RF: mom who used cocaine or other vasoconstrictive drugs
35
what congenital cardiac defect is common in Edward's syndrome?
VSD
36
mgmt ITP
observation regardless of the platelet count | if severe bleeding, give IVIG or steorids
37
what illness get a rash after giving ampicillin or amox?
Mono
38
tx gonococcal infxn in newborn
IM CTX
39
tx chlamydia infection in newborn
oral erythromycin
40
what skin lesion in newborns should be removed
nevus sebaceous because of concern for cancer
41
high direct bili in a 7 day old newborn dx?
biliary atresia | dark urine, pale stool, needs surgery
42
baby with respiratory distress with excess drooling
TEF | dx: place feeding tube and do xray
43
umbilical hernia associated with what?
hypothyroidism big tongue
44
2 week old infant with bilious vomiting, pregnancy complicated by polyhydramnios
intestinal atresia or annular pancreas | assoc with downs syndrome
45
3 day old newborn hasn't passed meconium? dx?
meconium ileus: dx/tx is gastrograffin enema Hirschsprung's: DRE --? explosion of poop, bx shows no ganglia
46
what don't you do to a newborn with hypospadias
circumsize
47
lead poisoning tx
Mild 5-44: no tx, repeat in 1 month Mod 45-69: give dimercaptosuccinic acid >69: Dimercaprol + EDTA
48
neonatal fever tx
amp and gent until 48hr cultures negative, add cefotax if suspect meningitis
49
Café au lait spots, seizures, large head, AD
NF
50
Mandibular hypoplasia, glossoptosis, cleft soft palate with FAS or Edwards
Pierre Robin sequence
51
Broad, square face, short status, self injurious behavior. Deletion on Chr17
Smith Magenis
52
Seizures, strabismus, sociable with episodic laughter. Deletion on maternal Chr15
Angelman
53
Elfin appearance, friendly, increased empathy and verbal reasoning ability. Deletion on Chr 17
Williams
54
2 yo with multiple ear infections, diarrheal episodes and pneumonias. No tonsils on exam
o X-linked agammaglobulinemia o Infections start a 6-9 months when maternal antibodies are no longer present o Labs: absence of B cells on flow cytometry, low levels of all Igs
55
17yo F with ↓ levels of IgG, IgM, IgE and IgA but normal numbers of B cells
``` o CVID (acquired) o Complication: increased lymph tissue→ increased risk for lymphoma ```
56
MC B cell defect: recurrent UTIs, diarrhea
o Selective IgA deficiency | o Complication: anaphylaxis reaction if given blood containing IgA
57
3 week old male with seizures, truncus arteriosus and micrognathia
o DiGeorge syndrome o Genetic defect: microdeletion on Chr22 o Candida, viruses and PCP pneumonia infections as a kid
58
Infant with severe infections, no thymus or tonsils, severe lymphopenia
SCID Bacterial, viral and opportunistic bugs MC is XLR. AR is Adenosine Deaminase deficiency Tx: peds EMERGENCY, need BMT by age 1 or death
59
3yo M child with recurrent swollen, infected lymph nodes in groin and staph aureus skin abscesses treatment?
Chronic Granulomatous disease XLR PMNs can ingest but not kill catalase + bugs (NADPH oxidase deficiency) Dx: Nitrotetrazolium blue (yellow means they have the disease) and new test is flow cytometry with DHR-123 Tx: daily bactrim and gamma interferon 3x/wk, BMT is curative but $$$
60
o 18mo M baby with severe eczema, petechiae and recurrent ear infections
o Wiskott-Aldrich Syndrome o Often present with prolonged bleeding after circumcision o ↓IgM, ↑IgA ↑IgE, slightly low IgG decreased platelets 2/2 decreased platelet production infections with encapsulated organisms
61
o Delayed separation of umbilical cord, recurrent bacterial infections, necrotic skin lesions, gingivitis
o Lekocyte adhesion defect o Neutrophilia without polymorphs in the infected tissue or pus o ↓CD18
62
: harsh systolic ejection murmur at L upper sternal border, single S2 -- Dx?
tetralogy surgery before 6 months is tx
63
14yo boy, starts out in 50% for height, in past 2 years is now between 5-10%
* Pathologic Short Stature | * Consider: craniopharyngioma (vision problems), hypothyroidism (TSH), Hypopit (check IGF-1), Turners (check karyotype)
64
MMR contraindication
neomycin or streptomycin allergy
65
when are live vaccines given
12 months MMR VZV HepA
66
are febrile seizures a contraindication to tDAP?
no!
67
Newborn cyanotic, O2 doesn’t improve
o Transposition of great arteries • MC: infants of diabetic mom • No assoc murmur
68
2yo who gets cyanotic and hyperpnea while playing, squats down
o Tetraology of Fallot • VSD and RA hypertrophy, overriding aorta, pulm stenosis • Murmur: harshe SEM + single S2 • Tx: O2 and knee-chest position, surgical correction
69
Bipolar woman gives birth to a child with holosystolic murmur worse on inspiration? Assoc arrhythmia?
o Ebstein anomaly o Tricuspid insufficiency 2/2 TV displacement into RV o Associated arrhythmia: WPW
70
Heart defect associated with DiGeorge syndrome. CXR shows ↑ pulm blood flow and bi-ventricular hypertrophy
o Truncus arteriosis o Eisenmenger develops early o Do surgery in 1st few weeks of life
71
Tx PDA
if not close by 1 week, tx with indomethacin or surgically close
72
Tx HOCM
BBs | CCBs
73
does PFO have an associated murmur?
NO
74
kid with failure to thrive in early childhood, what dx should you suspect
CF
75
o Precocious puberty, café au lait spots and multiple bone defects o
McCune Albright 5% of precocious puberty o assoc with hyperthyroidism, prolactin or GH secreting pituitary adenomas
76
tx Ca oxalate kidney stones
HCTZ
77
FH of kidney stones, what are they made up of
cystieine, can't resorb certain AA
78
MCD tx?
prednisone for 4-6 weeks | make sure immunized against pneumococcus and varicella sine infection is the most common complication
79
dx in a nephrotic patient who suddenly develops flank pain?
renal vein thrombosis 2/2 peeing out ATII, protein C and S Do a CT or stat US
80
MCC of sepsis in sickle cell kids
strep pneumo
81
tx acute chest syndrome
o2, abx and transfusion
82
tx sickle cell pt with stroke
transfusion NOT tPA
83
o 4mo pale baby, normal platelets, WBCs but hemoglobin is 4. Increased RBC ADA and low retics, triphalangeal thumbs
* Diamond Balckfan anemia | * Tx: corticosteroids, transfusions, stem cell transplant
84
o 18 mo baby with ↓ plt, ↓WBCs and profound anemia. Has café-au-lait spots, microcephaly and absent thumbs
* Fanconi Anemia * Dx: bone marrow shows hypoplasia, cytogenetic studies for chromosome breaks * Tx: corticosteroids, androgens, BMT
85
2 yr old baby with hyperactivity, impaired growth, abdominal pain and constipation
* Lead poisoning * Dx: venous blood sample, check lead level * Tx * >45 with succimer * >70 admit and tx with EDTA and dimercaprol * Screening: test blood levels between 12-24 mo if low SES, live in old house (
86
15 yo F with recurrent epistaxis, heavy menses and petechiae. ↓plt only
ITP tx: IVIG for 2 days then pred then splenectomy NO PLT TRANSFUSION
87
15yo F with recurrent epistaxis, heavy menses, petechiae, normal plts, ↑bleeding time and PTT
vWd DDAVP for bleeding or pre-op place factor VIII contains vWF if bleeding continues
88
7 yo M recurrent bruising, hematuria, and hemarthroses, ↑PTT that corrected with mixing studies
* Hemophilia | * If mild, tx DDAVP, otherwise replace factors
89
1 week old newborn, born at home comes in with bleeding form the umbilical stump and bleeding diathesis
* Vit K def * ↓ II, VII, IX and X * same in a CF kid with malabsorption * Tx: FFP acutely and vit K shot
90
HUS tx
NO PLATELETS TPN EARLY PERIOTNEAL DIALYSIS DO NOT HAVE ANX FOR BLOODY DIARRHEA
91
2 yr old with HTN, asymptomatic abdominal mass that doesn't ross the midline
Wilm's tumor best test: abomdinal CT, CXR for lung involvemnt tx: surgery, cehmo, rads
92
4 yo with jerking mvmts of the eyes and legs, bluish skin nodules and tender abdominal mass that crosses the midline
Neuroblastoma Dx: increased urine homovanillic or vanillymandelic acid
93
tx for otitis externia
topical cipro
94
tx Croup
rac epi neb | steroids
95
bronchiolitis tx
albuterol nebs no steroids
96
CXR hyperinflation with patchy atelectasis, dx?
bronchiolitis
97
tx pertussis
erythromycin for 14 days + same for kids in daycare and family members
98
who needs at VCUG
all males females 5 with 2nd UTI any pyelo
99
5 yo male initially with a cold 1 week ago, now presents with a limp and effusion in the hip, x rays are normal, ESR is 35, T 99.8, WBCs 10k Dx?
transient synovitis next best step: bed rest for 1 week + NSAIDs
100
Tx SCFE
surgical pinning
101
good and bad prognostic factors for juvenile rheumatoid arthritis
good: +ANA bad: +RF Tx in order NSAIDs MTX steroids
102
Tx KD
IVIG | ASA
103
tx febrile seizure
acetaminophen | no increased risk for epilepsy
104
6 month old brought in for multiple symmetric contraction episodes of the neck, trunk, extremities that occur in spells dx? tx?
infantile spasms common EEG finding: hypsarrhythmia: asynchronous, chaotic, bilateral Tx: ACTH (pred is 2nd line)
105
newborn with extended wrist, decreased grip, claw hand
Klumpke's palsy C8 and T1 should resolve on own also have ipsilateral horners
106
newborn with waiter's tip hand?
Erb-Duchenne palsy C5-6 grip intact, decreased moro
107
kids with CF who are less than 20 yo should get what ABx?
vanc bc staph is MCC of infections in CF pts who are under 20 years old
108
tx for acute bacterial sinusitis
augmentin
109
distress, dysphagia and drooling: dx and tx?
epiglotitis | tx = endotrach intubation in the OR
110
boy with 2d history of decreased appetite, neck swelling and irritability, keeps his head slightly rotated to the side: best step in mgmt?
x ray likely has acquired torticollis MC due to URIs, minor trauma, cervical lymphadenitis and retropharyngeal abscess need C spine films to rule out a fracture or abscess
111
kid less than 2 with wheezing, crackles, tachypnea, retractions and nasal flaring, dx and tx
bronchiolitis supportive tx watch out for apnea, esp in kids less than 2 months
112
angioedema due to what deficiency
C1INH--> elevated levels of edema producing factors C2b and bradykinin
113
dx inspiratory stridor that's worse when supine, crying or feeding and improves in the prone position?
laryngomalacia dx: flex laryngoscopy, shows omega shaped epiglottis - most will resolve on their own
114
what intervention for a breech presentation before week 37?
nothing, usually self correct before 37th week
115
workup for apnic spells? what associated with
CBC associated with iron deficiency anemia
116
how prevent intraventricular hemorrhage?
avoid preterm birth | corticosteroids
117
what does APGAR tell you?
how newborn tolerated labor | newborn's response to resuscitation
118
mental retardation, vomiting, athetosis, seizures and developmental delay over first few months? Dx?
PKU tx: low Phe diet
119
Mental retardation, direct hyperbili & jaundice ↓glc, cataracts, seizures Dx?
Galactosemia tx: no lactose for life
120
when worry about neonatal jaundice? workup?
Bili >12 direct bili >2 increases > 5 per day Coombs: (+) = ABO incompat or Rh (-) --> twin/twin or mom/fetus transfusion or G6PD, etc.
121
tx for pathologic neonatal jaundice
phototherapy, exchange transfusion if that doesn't work
122
what do we have to rule out with direct hyperbilirubinemia?
``` sepsis! galactosemia hypothyroid choledochal cyst CF ```
123
how dx and treat choanal atresia?
dx: can't pass a catheter, CT scan tx: oral airway, gavage feeding, surgical repair
124
tx respiratory distress syndrome
O2 therapy with nasal CPAP to keep alveoli open due to Surfactant def, can’t keep alveoli open.
125
38 wkLGA infant born by C/Sto an A2GDM has dyspnea/grunting, CXR is clear: dx? tx?
transient tachypnea of the newborn Lung fluid not squeezed out, retained Usually minimal O2 needed. Self-resolves in hours to days.
126
``` 41 wkAGA infant was born after ROM yielded greenish- brown fluid. *Next best step? *Complications? ```
Meconium aspiration syndrome Intubate & suction before stimulation Pulmonary artery HTN, pneumonitis
127
Newborn child with ambiguous genitalia. One month later has vomiting & ↓Na ↑K and acidosis. –MC Cause? –Definitive test? –Tx?
Congenital Adrenal Hyperplasia 21 Hydroxylase deficiency. (autosomal recessive) 17-OH progesterone before and after ACTH bolus Hydrocortisone and fludrocortisone (↑ doses in times of stress)
128
Complications of newborn hypoglycemia due to GDM? | Tx?
Neonatal seizure (always check glc!) Feed frequently if
129
Complications of newborn hypocalcemia?
Neonatal seizure (always check Ca!)
130
newborn with Hydrocephalus, intracranial calcifications and chorioretinitis dx/tx?
Toxoplasmosis. Tx w/ sulfadiazine + leucovorin.
131
Newborn with: Cataracts, deafness and heart defects (esp PDA, VSD), extramedullary hematopoeisis. dx? tx?
Rubella. No tx.
132
Newborn with Microcephaly, periventricular calcifications, deafness, thrombo-cytopenia and petechiae. dx? tx?
CMV. Tx w/ ganciclovir, but won’t prevent MR
133
newborn with: Limb hypoplasia, cutaneous scars, cataracts, chorioretinits, cortical atrophy dx? tx?
Congenital Varicella if mom infected 1st or 2nd trimester. If mom is exposed 5 days before –2 days after delivery, baby gets VZIG.
134
DOL 3-5, bilateral purulent conjunctivitis can cause corneal ulceration.
Gonococcal conjunctivitis tx w/ topical erythromycin and IV 3rd gen ceph.
135
DOL 7-14, red conjunctiva w/ mucoid discharge & lid swelling Dx? tx?
Chlamydia conjunctivitis tx w/ oral erythromycin. Complication is chlamydial pneumonia cough, nasal drainage, scattered crackles+ bilat infiltrates on CXR
136
Tx Turner's
estrogen replacement for secondary sex characteristics and to avoid osteoporosis
137
14 y/o boy, always been below 5% in height. Parents are tall & were “late bloomers”. Dx?
Constitutional Growth Delay | Bone age
138
short kid with short parents, bone age = real age, dx?
Familial Short Stature | Bone age = Real age.
139
what age does stranger anxiety begin
6 months
140
tx enuresis
behavioral tx DDAVP Imipramine (2nd line because of side effects)
141
immunizations at birth
Hep B
142
Loud S1 w/ fixed and split S2. Older child w/ exercise intolerance. dx?
ASD
143
Continuous machine-like murmur w/ bounding pulses and wide pulse pressure. dx? tx?
PDA Prematurity, congenital rubella syndrome If not closed by 1wk, give indomethacin or surgically close
144
MC complication of acute rheumatic fever?
Mitral stenosis
145
Asthma: pt has sxs twice a week and PFTs are normal?
Albuterol only
146
Asthma: If pt has sxs 4x a week, night cough 2x a month and PFTs are normal?
Albuterol + inhaled CS
147
Asthma: If pt has sxs daily, night cough 2x a week and FEV1 is 60-80%?
Albuterol + inhaled CS + long-acting beta-ag (salmeterol)
148
Asthma: If pt has sxs daily, night cough 4x a week and FEV1 is
Albuterol + inhaled CS + salmeterol + montelukast and oral steroids
149
Diagnostic criteria for diabetes?
Fasting glc >125 (twice)2hr OGTT (75g) > 200Any glc > 200 + symptoms
150
MC nephropathy?
IGA nephropathy, Berger's
151
best first test PSGN?
ASO titer
152
How tx Ca-oxalate stones
HCTZ
153
Treatment for kidney stones –Stones 2cm –Stones 5mm-2cm
Will pass spontaneously. Just hydrate Open or endoscopic surgical removal Extracorporal shock wave lithotropsy
154
Sickle cell kid with Proteinuria and increased creatinine+ recurrent UTIs?
Kidney infarcts due to sickled RBCs
155
Tx Thalassemia in kids?
Tx w/ transfusion & deferoxamine. Can see expanded medullary space target cells on smear
156
9 y/o F with Wilson’s disease developed fulminant liver disease. –1stfactor depleted? –2 factors not depleted?
VII, so PT increases 1st | VIII and vWF b/c they are made by endothelial cells.
157
TX HSP
Symptomatic treatment. | Can use steroids for GI or renal dz.
158
3 year old girl w/ a limp & left leg pain, T = 99.9, HSM, petechiae, & pallor. Cells are shown. Cells are CALLA and TdT + Dx? best test? tx? poor prog factors?
ALL Bone marrow biopsy >30% lymphoblasts VDP + CNS tx w/ intrathecal methotrexate 10, >100K WBC
159
14 y/o boy w/ enlarged, painless, rubbery nodes, drenching fevers, and 10% weight loss. –Best test? –And then? –Treatment?
Hodgkin Lymphoma Excisional biopsy. Staging CT or laparoscopy. (determines tx) Chemo + Rads. 90% cure if stage I or II
160
7 year old girl with non-productive cough and large anterior mediastinal mass on CXR. --Best test? –Treatment?
Non-Hodgkin Lymphoma Biopsy of mass, bone marrow bx for staging Surgical excision if abdominal tumor. Can use anti-CD20 if B-cell tumor. Rads for some.
161
2y/o w/ a fever to 105, 3 days later gets a pink, mac-pap rash on trunk arms and legs.
Roseola HHV6
162
2y/o w/ a low grade fever, lacy reticular rash on cheeks and upper body (spares the palms/soles) –Who is this bad for?
5th Disease/Erythema Infectiosum-Parvovirus B19 Preggos, sickle cell, thalessemia
163
Fine, mac-pap desquamating rash begins on chest and spreads to neck, trunk, & extremities+ strawberry tongue. Sore throat 1-2wks prior. –Treatment
Scarlett Fever, Group A strep PCN prevents rheumatic fever. (won’t help reduce changes of APSGN)
164
Cough, runny nose, fever macular rash begins behind ears & spreads down. Gray spots on the buccal mucosa. •Tx?
Measles Vit a + supportive care
165
Sore throat, joint pain fever pinpoint rash on the face and spreads down. Rose spots on the palate. •Complications?
Rubella congenital rubella syndrome
166
Baby with poor feeding. Vesicles in the mouth on palms and soles + rash on buttocks.
Coxsackie virus Hand-foot and mouth
167
16 year old M with swollen parotid glands, fever & HA. | •Complications?
Mumps(paramyxovirus) | Orchitis and sterility
168
6y/o kid from coastal NC, went camping. Had fever, myalgias, abdpain. –Complications? –Treatment?
Rocky Mountain Spotted fever. Rickettsia rickettsii Vasculitis and gangrene Doxy no matter what age
169
Inflamed conjunctiva and multiple blisters. Nikolsky’s+/ | –Treatment?
Staph Scalded Skin Syndrome From exfoliative toxin Tx w/ IV ox or nafcillin
170
``` 2 y/o w/ fever to 102, tugging on his right ear. Patient’s tympanic membrane is red and bulging. –Most sensitive dx test? –RF? –Treatment? –Complications? ```
Otitis Media Limited mobility on insufflation or air-fluid level ↓SES, Native Americans, formula fed, tobacco smoke, around kids Amox or azithromycin for 10days. If no improvement in 2-3 days, switch to amox-clav Effusion-place tubes if bilat effusion >4mo or if bilateral hearing loss.
171
A child presents w/ “muffled voice”, stridor and refuses to turn her head to the left.
Retropharyngeal abscess | I&D for C&S. GAS + anaerobes. 3rdgen ceph + amp or clinda
172
Indications for tonsilectomy?
>5 episodes of strep/year for 2 years or>3 episodes/year for 3 years
173
1 y/o w/ fever to 100.5 & “barking” cough and loud noises on inspiration. –Most common bug? –X-ray buzzword? –Treatment?
Croup Parainfluenza virus “steeple sign” Mist, epinephrine neb, steroids
174
``` 2 y/o w/ fever to 104 & drooling w/ intercostal retractions and tripod position. –Most common bug? –X-ray buzzword? –Next best step? –Treatment? ```
Epiglottitis H. Flu B only in unimmunized Strep pyo, strep pneumo, staph “thumbprint sign” Go to OR and intubate Anti-staph abx + 3rdgeneration cephalosporin
175
Specific findings for chlamydia pneumonia?
Staccato cough, eosinophilia
176
``` 9mo infant w/ runny nose, wheezy cough, T = 101.5, and RR = 60. Retractions are visible and pulse ox is 91%. –Most common bug? –CXR findings? –Treatment? –Who needs vaccine? ```
``` Bronchiolitis RSV. Confirm w/ swab Hyperinflation w/ patchy atelectasis Hospitalize if respiratory distress. Albuterol nebs. NO steroids ``` Palivizumab for premies, CHD, lung dz, immune dz
177
Tx pertussis
erythromycin for 14 days
178
UTI follow up in kids?
Test of cure to confirm sterility
179
``` 2y/o F w/ a 2 wk history of daily fevers to 102 and a desquamating rash on the perineum. She has swollen hands and feet, conjunctivitis and unilateral swollen cervical lymph node. –Other lab findings? –Best 1st test? –Treatment? –Most serious sequelae? ```
Kawasaki ↑plts (wk2-3), ↑ urine WBC, ↑LFTs, ↑CSF protein 2D echo and EKG. Repeat the Echo after 2-3wks of tx Acute = IVIG + high dose aspirin. Then aspirin + warfarin Coronary artery aneurysm or MI
180
More diffuse bone pain in a patient w/ petechiae, pallor and increased infections
Don’t forget bone pain can be presenting sx for leukemia
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Anytime you see a meningocele or myelomeningocele…
Do a head CT looking for hydro-cephalus. (Arnold Chiari II)
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Anytime you see an infant with a head circumference >95th%...
Consider hydrocephalus. Also bulging fontanelle, ↑DTRs, HA, vomiting.
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Noncommunicating hydrocephalus
Stenosis of CA, tumor/malformation near 4th ventr
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Communicating hydrocephalus
SAH, pneumoncoccal/TB meningitis, leukemia
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Infant with increasing head size, prominent occiput, cerebellar ataxia and delayed motor development. –Dx? –What will you see on CT or MRI?
Dandy-Walker malformation | Cystic expansion of 4th ventricle. Can see Agenesis of cerebellar vermis.
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8y/o w/ difficulty w/ balance while walking, no DTRs, bilateral Babinski and “explosive, dysarthricspeech”. –Most common cause of death?
Friedrich Ataxia AR, trinuc repeat HOCM --> CHF.
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2y/o w/ gait disturbance, loss of intellectual fxn, nystagmusand optic atrophy. Cresylviolet metachromatic staining. –Pathophys?
Metachromatic leukodystrophy | AR Deficiency of arylsulfatase A accum cerebroside sulfate
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12y/o w/ decreased school performance, behavior changes, ataxia, spasticity, hyperpigmentation, ↑K, ↓Na, acidosis. –Prognosis?
Adrenoleukodystrophy XLR | Death w/in 10 years
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9mo who had previously been reaching milestones starts to lag. Seizures, hypotonia, cherry red macula Pathophys?
Tay-Sachs XLR Def of hexosaminidase A accum GM2
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3mo infant lays in the “frog-leg” position,
SMA 1-Werdnig Hoffman Disease | Most die before age 2
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6y/o is brought in 2/2 “clumsiness” and frequent falls. The lower leg has decreased muscle bulk and appears “stork-like”. There are multiple small injuries on the hands and feet. You notice pes cavus and claw hand. –Dx? –Tests? –Treatment?
Marie-Charcot-Tooth Disease Decreased motor/sensory nerve vel, sural nerve bx. *CPK is normal Stablize ankles w/ surgical fusion. Usually normal lifespan and most remain ambulatory.
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tx meningitis 0-4 weeks?
Amp + cefotax
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tx meningitis 1 year to 50 years?
Vanc + CTX
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pt presents with recent URI and neovascularization of cornea: dx?
trachoma 2/2 Chlamydia dx: giemsa tx: topical azithro or tetracycline