Board Vitals #3 Flashcards
1
Q
Patient with two different colored eyes, patches of hypopigmented skin, confluent eyebrows and broad nasal bridge needs what additional test?
What is this?
A
Needs hearing tested (50% have congenital sensorineural hearing loss) Waardenburg syndrome (gray or white forelock)
2
Q
Most brachial plexus birth injuries occur to which portion?
A
Upper trunk
Most unilateral and from stretching of C5-6 with decreased moevement of affected arm; upper arm adducted and internally rotated
3
Q
What infants should receive Palivizumab?
A
born <29/0
born <32 with BPD until 12 months old
4
Q
Infant presents with diffuse maculopapular rash, started on neck and trunk and spread to arms and face. Had fever 3-4 days before rash
A
Sixth disease or roseola infantum from HHV-6
5
Q
Parvovirus B19 can lead to this serious complication, esp in SS patients
A
Aplastic Crisis
VERY infectious
6
Q
When are patients with classic Parvo B19 (erythema infectiousum) most contagious
A
Get fevers, nausea, HA and coryza then slapped cheek facial rash; once rash appears no longer infectious
7
Q
Neurocutaneous disorder with telangiectasia of skin, GI, retina and mucous membranes.
What chromosome and gene are involved?
How do these pts present?
A
Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu
Auto Dominant
mutationon HHT1 gene on chrom 9
See epistaxsis, GI bleeds, telangiectasia on lips, oral mucosa and fingertips
8
Q
Predisposes individuals to freckling and malignancies when exposed to UV light. Get basal cell, melanoma and systemic tumors as well as progressive hearing loss, chorea, ataxia
A
Xeroderma pigmentosum
9
Q
What gross motor milestones are present at 9 months?
A
Throw ball overhead, jump, walk up and down stairs one at a time
Stack 6 blocks, copy a line, point to a picture
10
Q
What patients require immediate referral to a burn center?
A
3rd degree burns (full thickness; white, dry and painless), >10% body surface, or sensitive areas (face, perineum, hands, feet, mouth)
11
Q
What is the Parkland formula, when do we use it?
A
Fluid management for burns >15% of body surface Add EXTRA (4ml/kg) _ % body burned to maint fluids and gie 1/2 over the first 8 hrs from time of burn.
12
Q
Most common pediatric arrhythmia
Why does this occur?
A
Paroxysmal SVT
From AV and AV node reentrant tachycardia ~ narrow complex with rapid onset
13
Q
Tx for SVT
A
vagal, adenosine and AV nodal active agents
14
Q
An event that results in death, permanent harm or severe temporary harm
A
Sentinel event
15
Q
A event, preventable or non that caused harm to patient as result of medical error
A
Adverse event
16
Q
Patient is diagnosed with Developmental hip dysplasia. What is treatment if
<6 months
>6 months
A
<6months treatment is Pavlik harness (splint to prevent hip extension and limits adduction)
>6 months is closed or open reduction with hip cast
17
Q
What imagin modality do we use if we suspect DDH?
A
Ultrasound if >2 weeks but <6 4 months
Hip XRAY after 4 months
18
Q
What children need imaging for DDH?
A
All female breech (get US at 6 weeks)
Consider boy breech or in history of first degree relative
IF 2 consecutive exams that are concerning or equivicol
ANY UNEQUIVICOL exam
19
Q
Deficiency presents with weakness, cerebellar ataxia, partial opthalmoplegia, muslce aches and altered sensation in fingers and toes
A
Vitamin E
only one to cause spinocerebellar degeneration
See if issues with fat absorption
20
Q
Presents with irritability, excessive startle response and seizures not responsive to AEDs
A
Pyridoxine or B6 deficiency
21
Q
What is PPX for people in contact with someone who has N.men?
A
1 dose IM CTX or 4 doses PO Rifampin over 2 days OR once dose of Ciprofloxacin orally
22
Q
Patient with devo delay and macrocephaly with cherry red spot and exaggerated startle reflex. No organomegaly with decreased tone at 9 months
Disease and pathology?
A
Tay Sachs (no organ involvement) Sphingolipidses from deficiency of Hexosaminidase A enZ
23
Q
Axillary freckles, neurofibromas, cafe au lait macules (6 or more and HYPERpigmented) are concerning for?
What screening should be done
A
Neurofibromatosis
Screen for opthalmologic exam for optic pathway glioma
24
Q
Facial angiofibromas, shagreen patches, ash leaf macules and subependymal nodules are associated with
A
Tuberous sclerosis
25
What are shagreen patches and when do we see them?
Fleshy, light pink, have raised and 'orange peel' appearance. On trunk or lumbrosacral area
Seen with Tuberous sclerosis
26
Patient with isolated hematuria on repeat urinalysis with negative culture. Next step?
If history is neg for infection, trauma or FHx, get urine calcium and creatinine
Uca:Ucr ratio >0.2 suggests hypercalciuria
27
When starting a patient on prostaglandins or PGE1 to keep PDA patent, what else needs to be done?
Intubate; PGE's can cause central apnea
28
10 month old with croupy cough, SpO2 in high 80s wit fever and inspiratory stridor. Normal breath sounds and clear. Was given decadron earlier in the day without effect
Bacterial tracheitis; inflammation of larynx, trachea and bronchi. Seen more in peds
Stridor or barky cough, high fevers and hard to breath
29
What does CHARGE stand for
```
Coloboma
Heart defects
Atresia of chonae
Retardation of growth and devo
GU anomalies
Ear anomalies or deafness
```
30
```
Patient with Rickets from Vit D defiency has
_____ 25 OH Vit D
____ PTH
_____Calsium
____ alk phos
```
Low Vit D
Elevated PTH
Low Ca
Elevated Alk phos
31
When do we get Tylenol level for accidental or intentional ingestion?
When can we give charcoal
4 hours after
| Gastric decotamination with charcoal can be done w/i 4 hours post ingestion
32
When do we give NAC for tyenol ingestion
Above tx line on Rumack-Matthew nomogram
ingestion >150mg/kg
pt with delayed presention and serum tylenol >10mg/L
33
Pt with this disease can present months after birth with albinism, musty odor, stunted growth, devo delay and microcephaly
PKU
34
Accumulaiton of glucocerebrosides in tissue, pt present with splenomegaly, hepatomegaly, thrombocytopenia, osteopenia, bone pain
Gauchers; lysosomal dx
35
When would you order a total CH50 assay?
Concern for complement pathway pathology
| See recurrent sinopulmonary infections, recurrent neisserial infections and issues with MAC
36
Lab abnormality seen with pyloric stenosis
HYPOchlroemic HYPOkalemic metabolic alkalosis
37
Most common cause of hyperchloremic metabolic acidosis
RTA
| If associated with hypoKalemia then its type 1 or 2
38
When do we see hyperchloremic hyperkalemic metabolic acidosis
RTA type 4
39
6 year with left sided limp. Had a URI 4 days ago. Left hip w/o redness or erythema and antalgic gait with hip abducted and externally rotated. Most likely diagnosis and treatment
Toxic synovitis and tx with NSAIDS
40
What are the indications for starting insulin therapy in diabetic patient
Patients with hA1c >8.5% need to start or if hx of DKA
41
All streptococcus organisms are gram_____ and are in ____ and ____
gram positive
are in pairs and chains
Gram staining = BLUE
42
Which strep organisms are alpha hemolytic
viridans and pneumonia
43
Which strep organisms are beta hemolytic
Group A = pyogenes
| Group B= agalactiae
44
You have a patient that is strep positive with sympotms. Known PCN allergy. What do you treat with?
Erythromycin
OR
Clindamycin
45
Patient presents with peritonsillar abscess. What is treatment
Drain and Tx with IV antibiotics: Clinda and amp-sulbactam to cover for anerobes--> amox-clavulanate
46
What is the correct order of puberty in females?
Thelarche--> Pubarche--> Growth spurt 1-2 years before--> Menarche
47
Hyperbili due to Rh incompatibility is from ____
Antibody in mom
| anti-D IgG
48
What do we give to tx overdose due to beta blocker with symptomatic bradycardia, resp depression, hypoT and hypoglycemia
Glucagon and Atropine
49
What is the MOA of aminogycosides and which organisms are they effective against?
Binds aminoacly site of 16S rRNA within the 30S subunit to block protein synthesis
good for anaerobes and gram negatives
50
Patients ___ to ___ require 2 doses of flu vaccine the first time they receive spaced ___ weeks apart
6mo to 8 years
| seperated by 4 weeks
51
How do you calculate midparental height for male
Moms + Dads ht in cm + 13
Divided by 2
*ht should be 5 cm above or below
52
How do you calculate midparental height for female
Moms + Dads ht in cm - 13cm and can be 5 cm above or below this number
53
MOA of Clonidine
alpha-2-adrenergic receptor stiumulator--> reduced sympathetic outflow
-decrease in peripheral vascular resistance, renal vascular resistance, and decreases HR and BP
54
SE of Clonidine
worsen or cause depression, fatigue, dizzy, dry mouth, HA, abdominal pain,hypoT
55
Which drugs can be given through an ET tube?
```
LEAN
lidocaine
Epinephrine
Atropine
Naloxone
And surf
```
56
2 day old with acute onset poor feeding, fTT, vomiting and lethargy.
Labs notable for ketoacidosis, elevated anion gap and moderate hyperammonemia.
Cause?
Organic acidemia
57
Patient with ketosis, elevated ammonia, anion gap and thrombocytopenia. Has sweaty feet and a seizure. 1 week old.
Organic acidemia
| *Isovaleric Acidemia
58
What causes organic acidemias?
| Lab abnormalities?
Enzyme deficiencies lead to difficulty breaking down organic acids from AA and FA.
See anion gap MA, Ketosis, Hyperammonia (often)
*present by DOL2
59
What is Tx for organic acidemias?
Limit protein and give L carnitine to repleate depleted stores, IV glucose and ammonia scavengers
60
How do MMA and proprionic acidemia present?
Poor feeding, vomiting, dehydration, tachypnea and hypotonia. Have anion gap MA, ketonuria, hyperammonimia.
Get urine organic acids and see elevated MMA or PA
61
Treatment for MMA?
Vit B12
62
4 year old presents with fevers to 104 and oral lesions in the oropharynx with decreased PO intake. No other skin lesions.
Herpangina from Coxsackie virus
63
Patient presents with fever and new onset seizure. EEG shows periodic lateralizing epileptiform discharges. If you obtain CT what area of the brain is likely infected?
What is treatment?
HSV encephalitis
Temporal lobe involvement
Tx stat IV acyclovir and get LP for PCR testing
64
5 year old with hearing loss and delayed growth. Normal at birth. History of strokes and labs notable for elevated lactate and uric acid. Has frequent headaches and is acutely encephalopathic
MELAS
Mitochondrial Encephalopathy Lactic acidosis ad Stroke like episodes
Confirm with gene analysis
65
Patient's with Hashimoto Thyroiditis present with ___ TSH ____ T4 and elevated ____ antibody
HIGH TSH
LOW T4
elevated TPO or thyroglobulin
66
Used when doing case control study to compare exposed and non-exposed group to determine probability of outcome of interest between the two
Relative Risk
| Divide probability of outcome of interest in: Exposed/Unexposed
67
Used in Case Control studies
| Tells you chance of having a disease given an exposure
Odds ratio
| Divide odds of disease after exposure/odds of disease without exposure
68
Patient with respiratory alkalosis, metabolic acidosis and ear ringing, they have elevated HR and RR.
Dx adn tx
ASA or salicylate toxicity
Tx with urine alkalynization
may be able to give activated charcoal if acutely presented
69
Any patient with JIA that is 6 years or less and ANA positive needs to have...
optho eval for uveitis. Done w/in 1 month of diagnosis and then every 3 months for 4 years.
Then every 6 months for 3 years then yearly.
70
Features present in Prune belly syndrome (triad)
Deficient devo of abdominal muscles, GU anomalies and cyrptorchidism.
Often prostatic and ureteral hypoplasia--> obstructive uropathy in utero leads to megauretery/hydronephrosis
71
What are the HACEK organisms?
Haemohilus aphrophilus
Actinobaccilus
Cardiobaterium hominims
Eikenella
Kingella
*Fastidious gram negative bacilli; take up to 5 days to grow
*resist amox, tx with 3rd gen ceph plus gent
72
Marfanoid habitus with poster/downward displaced lens and devo delay
dx
tx
Homocystinuria
Autosomal recessive
cystathione-B-synthase deficiency and see elevated methionine levels and homocysteine
Tx: cysteine
73
Patient with several days of explosive diarrhea and persisetant diarrhea with bloating that is painless and no blood or mucous with hyperactive bowel sounds. Most likely infectious cause and treatment
Giardia duodenalis
Tx Metronidazole
Dx with stool microscopy or antigen
74
Patient with recurrent staph and strep infections and oculocutaneous albinism.
Cause and genetics
Chedaik Higashi
Auto-Recessive
abnormal neutrophil function; fusion of primary and secondary granules--> giant granules in neurtrophils, lymphocytes, plts and melanocytes
75
Patient with direct hyperbili at 7 days of life. Paucity of bile ducts present. What cardiac and skeletal anomalies as well as facial features are seen
Pulmonic stenosis
butterfly vertebrae
hypertelorism and triangle facies
76
Presents in teens that are during sports with sudden and intense muscle contracture and seen in kicking, springing, jumping and frequently misdiagnosed.
Pt present with posterior thigh pain and pain at hip
Avulsion fracture; seen frequently at anterior superior iliac spine
Heal over 4-6 wks with rest, ice, and non weight bearing
77
Infectious diarrhea that causes fever, body aches then abdominal pain, vomitting and bloody diarrhea.
Complications include HUS, seizures, bactermia, reactive arthritis.
Dx and tx
Shigella
| Tx supportive
78
Associated with progressive non-atherosclerotic bilatearl stenoses of distal internal carotid arteries and trunks of anterior and middle cerebral arteries. MC in Japanese girls
Moyamoya
| See 'puff of smoke' on angiogram
79
Patient is newborn presents with hypocalcemia and TOF. What is the syndrome?
What cell line is affected?
How do we diagnose?
DiGeorge; 22q del from abnormal 3/th brachial pouches
T cell immunity defective bc no thymus (thus no maturation)
Dx with FISH with DNA probe
80
What is cause of Down Syndrome
meiotic nondisjunction
81
Patient presents wtih recurrent S. PNA infections as well as H influenza and PSA.
Arrest of B cells in Pre-B cell state.
X-link agammaglobulinemia
| mutationin Btk gene on Xq22
82
Symptom of deficit of voluntary motor function in pat w/o anatomic of physiologic basis. Present with impaired coordination, paralysis, vision changes, nonepileptic seizures
More common in females
Conversion disorder
83
Intentional feigning of physical or psychological symptoms without secondary gains (no financial or legal gain) and mimics a known syndrome
Factitious disorder
84
Systolic crescendo-decrescendo murmur, II/VI in healthy patient
Vibratory or Stills murmur; benign
85
Low pitched mid-diastolic mrumur at cardiac apex
Mitral stenosis
86
In prerenal acute kidney failure renal tubules respond to decreased perfusion by conserving water and sodium so that urine osmolality is > ______ and urine sodium is
urine osmolality is >400-500
| urine sodium is <10-20mEq/L and FENa is < 1%
87
Most children double their birthweight by _____ and triple their weight by _____
double by 4-5 mo
triple by 12 mo
quad by 2 years
88
Teen with recurrent Sinus infections and two episodes of diarrhea from giardia. Normal CBC, LFT.
What test do you want, what do you suspect if its CVID
get Ig levels
See low GAM (IgG, A, M)
picture teenager with big (GAMS = thighs) with runny nose running to get her Izs once again!
89
Patient with recurrent infections, eczema, male. What do you see on cbc
thrombocytopenia with small platelets
| wiskott aldrich
90
Immune system is made up of the INNATE immune system =
Innate = neutrophils, eosinophils mono/macrophage, complement, cytokine/chemokines
91
Immune system is made up of Innate and ADAPTIVE which is
```
Adaptive = Humoral which is B cells (from plasma cells) to make antiBodies
Cellular = T cells for cell mediate immunity against virus/fungus/intracellular organisms
```
92
What test will diagnose a humoral or cellular immunity defect? (issue with B cells or T cells)
Lymphocyte phenotyping by Flow cytometry
93
Patient with recurrent derm and pulm infections, gets lots of abcesses and has bone and joint infections likely has issue with...
Neutrophil defect (innate)
94
Patient with recurrent bacterial infections with encapsulated organisms and severe or recurrent resp tract infections has issue with...
Complement defect
95
Patient with recurrent pyogenic infections with encapsulated organisms, otitis, sinusisits, PNA, conjuctivitis and decreased levels of immunoglobulins has issue with..
Humoral immunity defect
96
Patient with reuccrent infection with opportunistic or less virulent organisms like viruses, has growth retardation, diarrhea, FTT and poor survival past childhood has issue with...
Cellular immunity defect
97
Depression screening and risk based assessment for drugs/EtOH begins at age ____
11
98
At what age do we screen for dyslipidemia?
risk based age 2 if FHX
| screen at 9-11 yo and again 18-21
99
Risk based screening for STIs/HIV occurs as early as ____ with mandatory test at ____
11
| 16-18
100
What are complications of pinworms and what is treatment?
UTI and vulvovaginitis
| Tx albendazole, mebendazole or pyrantel
101
Nephrotic syndrome is often accompanied by what change in CBC
thrombophilia
102
What injuries are sustained when children wear a seatbelt with just lap belt?
Transverse abdomimal wall contusion, lumbar Chance fracture (in upper lumbar spine) visceral trauma like jejunal rupture or liver lac
103
Patient presents with pityriasis rosea. What is expected course and treatment?
Rash resolves on own. No tx needed, could do topical steroid if really itchy
104
How frequently do we screen thyroid function in Trisomy 21?
birth, 6 mo 12mo and annually
105
Patient with recurrent thrush and fevers has issue with ___ part of immune system and has issue with what ene?
T cell immunity issue; Chronic mucocutaneous candidiasis or CMCC
Defect of AIRE gene or autoimmune regulatory gene
106
Seizure dx that starts with myoclonic jerks and progresses into GTC, worse with sleep deprivation. What is treatment and diagnosis?
Juvenile myoclonic epilepsy
Sz in AM, is hereditary and is lifelong condition.
Tx valproic acid`
107
What is the MC testicular mass seen in pre-pubertal boys?
Teratoma
108
Infants less than 1000 grams are at risk of ____ and should have this type of screenign done.
IVH
head US
70% will devo grade IV IVH
109
Weight of <1000 grams is linked to this complications long term
neuro impairment and risk
110
Normal hearing has a threshold of ___ to ___ dB
0 to 20 dB; anything abnormal on screening should be referred to audiology
111
Cleft in vertebral column with defect in skin, exposed meninges and spinal cord.
Myelomeningocele; spina bifida
| MC NT defect
112
Fluid filled gliosis lined cavity within the spinal cord and can lead to progressive scoliosis, spasticity and weakness of extremities
Syringomyelia
113
What are the requirements for BPD diagnosis?
O2 requirement at 28 days postnatal or 36 weeks postmentral age.
RF: <1250g, maternal smoking, IUGR, mechanical vent, infections
114
What are pathological findinds on lungs of BPD infants?
decreased septation, alveolar hypoplasia, increased elastic tissue formation, thickened interstitium.
115
What is Bornholm syndrome? Who gets is and what is infection??
fever, paroxymal spasms of chest and abdominal muscles.
Causes are Coxsackie B virus
See in summer time; often adolescents.
116
Induration of > ___mm indicates positive results even if BCG vaccine.
Assuming neg CXR, what is treatment for latent TB?
>15mm
Rifampin daily for 4 months
Isoniazid + Rifampin weekly for 3 months or
Isoniazid daily for 9 months
117
Patient is diagnosed with active TB (CXR was +), what is treatment?
RIPE
| Rifampin, isoniazid, pyrazinamide and ethambutol for 2 months then Isoniazid/rifampin for additional 4 months
118
Anabolic steroids have ___ feedback on hypothalamus
| Leads to ____ LH and FSH secreation
NEGATIVE
LOW LH and FSH secretion
= small balls, azoospermia and gynecomastia
119
MOA of clonidine
| Uses of Clonidine
central alpha adrenergic agonist; decreases peripheral resistance
Used for HTN but also can work for insomnia, ODD, tics and opioid withdrawl.
120
SE of clonidine
HypoT, irritability, dysphoria, parasomnias
121
One year old with fatty stools and FTT, ANC of 600 with hypochromic, microcytic anemia and low levels of trypsin, lipase adn colipase. Low Ig's and recurrent bacterial infections
Dx
Schwachman Diamond syndrome
| Auto Recessive
122
What do we see in Swachmann Diamond sydnrome
exocrine pancreatic insufficiency (fatty stools)
Bone marrow dysfuncion( low ANC, anemia)
Recurrent infection with los Ig
123
Zoonotic disease that causes fever, body aches--> cough and SOB with pulmonary capillary edema.
See thrombocytopenia, leukocytosis, elevated crit and immunoblasts
Hantavirus
| Confirm with serology
124
Patient with this tumor present with abdominal mass, constipation, proptosis, periorbital ecchymoses.
Can met to bones
Neuroblastoma
125
Disorder in newborns, see FTT, hepatomegaly, direct hyperbili, hypoglycemia and delayed healing of umbilicus. Smell like cabbage
Tyrosinemia
| AutoRecessive from fumarylacetoacetate hydrolase deficiency
126
Causes black urine when exposed to air, can see heart issues, kidney stones, prostate stones
Alkaptonuria
| Auto Recessive
127
Infant with FTT, lethargy, jaundice and coagulopathy. High risk for salmonella sepsis and cataracts
Galactosemia
128
Dog bites needed to be treated with ____ aBx when they occur to what areas?
Amoxicillin-clavulanate
To face, hands, genitals
MC organism = pasteurella
129
Naegleria fowler is an amoeba seen with transplant patients, causes meningoencephalitis. What is treatment
Miltefosine with medically induced hypothermia
130
What is the natural progression of the atopy march?
Eczema--> food allergies--> allergic rhinitis--> asthma
131
What is the gold standard to diagnose asthma
Methacholine challenge; has a high negative predictive value
It directly stims airway smooth msl receptors.
Pre/post spirometry is recommended initial screen in kids >5yo
132
What is the relationship between food allergies and eczema?
Food allergies do not cause eczema; thus do not routinely test! only test food allergies if IgE mediated reaction has occurred.
133
Gram negative sepsis can lead to shock and release of endotoxins which can cause...
DIC
| see coag cascade triggered--> thrombi and fibrin deposit in small vessels leads to schistocytes
134
Class of chemo drugs that are cardiotoxic
Anthracyclines; doxorubicin
135
Cells occur in ESRD and liver disease as well as pyruvate kinase deficiency
echinocytes or burr cells
136
tear drop cells that act as a marker for bone marrow infiltration and fibrosis
Dacrocytes
