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1
Q

infection often occurs after the ingestion of dirt contaminated with dog feces; the stool contains eggs produced by adult worms
Affects GI tracts, lungs, eyes
Can see crackles on lung exam, Hepatomegaly
Seen with little kid and new pet

A

Toxocara canis

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2
Q

is a condition that usually involves the 2nd costochondral cartilage on either side and is always associated with visible swelling and, in many cases, with erythema and warmth.

A

Tietze syndrome
Tx NSAIDs
Swelling makes different from costocondritis

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3
Q

Pts that require prophylaxis for SBE

A

patients with a prosthetic heart valve (including homografts and transcatheter implanted valves), a history of previous endocarditis, a history of complex unrepaired cyanotic congenital heart disease, or a systemic-to-pulmonary artery shunt or conduit. patients who have undergone repair of a congenital heart defect with prosthetic material within the previous 6 months or who have residual defects in close proximity to prosthetic material (i.e., patch) following repair of a congenital heart lesion. Patients who have undergone heart transplantation and have valve dysfunction require SBE prophylaxis.

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4
Q

What procedures require SBE prophylaxis

A

All dental procedures that involve manipulation of gingival tissue or perforation of the oral mucosa require SBE prophylaxis. SBE prophylaxis is also recommended for respiratory procedures that involve incision or biopsy of the respiratory mucosa including: tonsillectomy, adenoidectomy, or bronchoscopy with biopsy.

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5
Q

Describe Kostmann syndrome

A

Kostmann syndrome, children have myeloid arrest. Their myeloid stem cells are insensitive to G-CSF and fail to mature. They have no reserves, similar to a post-bone marrow transplant recipient, and are therefore extremely vulnerable to their own bacterial flora.

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6
Q

Cause of death from direct contact to chest

A

Sudden cardiac death secondary to ventricular fibrillation following direct precordial trauma—from, for example, a fist, baseball, lacrosse stick, or hockey puck—is known as commotio cordis

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7
Q

is an arrhythmic event (ventricular fibrillation) that occurs when a nonpenetrating blow to the chest causes a sudden increase in intracardiac pressure. If the trauma occurs during a narrow window of the cardiac cycle, a 10- to 20-ms interval during repolarization just prior to the peak of the T wave, then ventricular fibrillation may result.

A

Commotio cordis

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8
Q

Risk with ingestion of caustic agents

Management?

A

patient is at risk for necrosis of the esophageal and gastric wall due to the ingestion of sulfuric acid and should undergo endoscopy 12–24 hours after ingestion. Caustic ingestions can be acid or alkali. Caustic ingestions may be associated with dysphagia, burns in the mouth and throat, swelling of the throat, drooling, vomiting, esophagitis, and gastritis;
Endoscopy, NPo Iv fluids, obs

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9
Q

Patients ____have recurrent episodes of angioedema, especially of the skin, GI tract, and upper airway. is an ____ disorder caused by a low or non-functional C1 esterase inhibitor

A

Patients with hereditary angiodema an autosomal dominant disorder caused by a low or non-functional C1 esterase inhibitor

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10
Q

causes susceptibility to recurrent neisserial infections.

A

C8 deficiency (terminal complement deficiency) causes susceptibility to recurrent neisserial infections.

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11
Q

When does immunocompromised patient get varcilla immunoglobulin?
what is tx for active infection in this individual?

A

IG in patient exposed to varicella w/o symptoms, goal w/in 96 hours
Tx for active infection is IV acyclovir

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12
Q

Patient with scattered flat hyperpigented lesions on body, headache and scattered dark spots in eyes. concerning for?

A

NF 1, loss of function mutation; have cafe au lait, axillary freckling, Lisch nodues

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13
Q

Patient with seizures, intellectual disability and intracranial calcifications with scattered cutaneous lesions

A

Tuberous sclerosis

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14
Q

Patient with bilatearal acoustic schwannoma, early onset cataracts and meningioma or ependymomoma

A

NF2

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15
Q

What is the danger of using inhalants?

Tx?

A

Peak 7th to 9th grade, most are hydrocarbons or NO = CNS depressants.
Complications are N/V, ataxia, risk of pulmonary aspiration and can have cardiac dysrhythmia
Manage with supportive cares

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16
Q

Tx for pinworms

Who should get treated?

A

Tx albendazole, mebendazole and wash all bedding and clothes

Tx the family of index patient

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17
Q

What is the incubation period for varicella?
When are you most contagious?
MC complication?

A

Incubation: 10-21 days
Contagious 48 hrs prior to onset of rash until all lesions crust over; attack rate >90%
secondary skin infection MC complication

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18
Q

MCC of seizures in term newborn?

A

hypoxia, likely from intrapartum or antepartum asphyxia

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19
Q

Infant can sit without support, pull to stand, has 3 finger pincer grasp, bangs blocks, makes 2 syllable sounds and imitates speech sounds, says mama, dada

A

9 months

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20
Q

Child can stand alone, walk, put block in cup, imitate others, say mama, dada intentionally, 1-2 words and follow simple instructions, sip from cup

A

12 months

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21
Q

Can run well, walk backwards, walk up steps with one hand held, kick a ball, stack 4 blocks, use a spoon and fork, engage in pretend play and vocabulary of 10-25 words

A

18 months

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22
Q

Child can kick ball, throw ball overhead, jump and walk up down stairs one step at a time, stack 6 block, copy a line, point to pictures and follow 2 step command and put 2 words with vocab of 50 words

A

24 months

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23
Q

what is the triad of sturge weber sydrome?

A

facial port-wine stain, ipsilateral meningeal capillary malformations, ipsiliateral cerebral cortical capillary malformations

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24
Q

Define bulimia and the treatment

A

binge eating and inappropriate compensatory behavior to prevent gain: vomitting, laxatives, diuretics or fasting/exercising.
Tx: CBT and fluoxetine (if no concern for bipolar)

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25
Child with devo delay, micropenis and BL cryptorchidism, issues feeding at birth and low tone with FTT. Few years later, insatiable appetite and obese with small hands and feet. Inheritance?
Prader willi | Dele 15q11 from paternally derived chromosome
26
Patient with delayed motor milestones, low tone, macrocephaly and large testicles Dx and inheritance?
Fragile X | X linked from expansion of CGG in FMR1 gene
27
Child with small penis and testis, normal tone, normal devo.
Klinefelter syndrome | XXY with male phenotype
28
Slow growth, high pitch cry in infancy, mental deficiency and microcephaly. Most with IUGR, low BW and hypotonia with downslanting palpebral fissures
Cri du Chat | partial del of chrom 5
29
What does EKG with hyperK look like? | What treatments are given and why?
Tall peaked T waves, widening of QRS complex Goal shift K from serum --> intracellular Insulin is tx; this does that... glucose given to prevent hypoglycemia with insulin Calcium gluconate to stabilize cardiac membrane and inhaled Beta-2 (albuterol) to drive K into skeletal muscles May need dialysis
30
What is nursemaids elbow? Classic presentation? How to treat it?
Subluxation of the radial head or annular ligament displacement. seen 6mo to 5yo Pt refuses to move arm, holds in flexed and pronated position Tx: do not need imaging, apply pressure to radial head while supinating the forearm
31
patient with family history of CAD early onset, has lesions on extensor surfaces of elbows, knees and has LDL >500. Dx? Genetics? Tx?
Familial homozygous hypercholesterolemia disorder of lipoprotein metabolism = elevated serum LDL = increased risk for CAD Dx clinically plus FHx and genetics; worse corse if homozygous Tx with aggressive cholesterol reduction
32
ORder of events in male puberty
``` Testicular/scrotal enlargement Pubic hair/Axillary hair Enlargement of penis 6mo later Growth spurt 1-2 years later Facial hair, voice changes at end ```
33
Causes atypical PNA as well as formation of cross-reacting IgM antiB against RBC membrane--> leads to agglutination in cold areas of body (ear, hands, toes,nose) and potential erythrocyte hemolysis in liver and spleen
Mycoplasma PNA
34
Patient is 5 mo old, presents with fever, dehydration and hypoxia to 90%, CXR with diffuse pulmonary infiltates. Labs with elevated IgM and decreased IgA and IgG Dx? Cause? Genetics?
CD40 ligand deficiency--> leads to humoral immunodeficiency with elevated IgM, low other Igs from imparied interaction btwn T cells and other cells expressiong CD40; can do isoform switch from IgM to other classes X linked, male predominance B cell count usually unaffected
35
Pt with CD40 deficiency present with what illnesses? Why?
recurrent respiratory with encapsulated bactrial: s.pna, h.influenza and opportunistics like histo of PCP see elevated IgM, low G and A bc can't class switch from M to other Ig's See GI and pancrease malignancies later in life
36
form of humoarl immunodeficiency with decreased levels of 2 or more immunoglobulins
CVID or common variable
37
What are signs of pulmonary arterial hypertension? | What medications should we put patients on?
See elevated BNP from RA dilatation from elevated RV pressures. Hear a single or loud S2 with hepatomegaly Tx with phosphodiesteraese-5 inhibitor; Tadalafil
38
What is the danger with a sulfonylurea ingestion? | What is the treatment?
'one pill can kill' inhibit ATP-sensitive K channels in pancreatic beta cell= increase in insulin release 1/2 life of 10 hours with peak at 2-6 hrs Tx with 2 to 4mL per kg of D25W OR 5 to 10ml/kg of D10W ~ 0.5 to 1.0g per kg of dextrose admit for overnight observation
39
When and why do pregnant women get Tdap vaccine?
Get during 3rd trimester | For neonatal immunity; pass along antiB to neonate
40
Pt with 2 our of 4 genes abormal in alpha thal. | What is disease and are there symptoms?
2/4 is alpha thal MINOR | Sx are mild microcytic anemia
41
Pt with 3/4 genes mutated controlling alpha chain. | Disease?
Hemoglobin H | have microcytic anemia and mild hemolysis
42
6yo Pt presents with frothy urine, + proteinuria and casts. Recent skin infection. Bx shows effacement foot processes Disease? Changes to kidney?
Minimal change | no change to glomeruli
43
What do you see on kidney bx in patient with IgA nephropathy? When do patients have flaires?
NephRITIC syndrome see episodic hematuria w/in 1-2 days of URI See mesangial proliferation
44
What is the most serious form of renal issues in patient with lupus?
Diffuse proliferative glomerulonephritis = Nephritic get immune complexes all over==> thickened capillary wall and see 'wire looping' on microscopy with subendothelial immune complexes
45
What do you see on EM in patient with acute PSGN? | Why does this occur?
complent activated via classical path--> injury to tissues via complement activation and see hypocomplemntiemia and granular deposits of IgG and complement on GBM.
46
I can hold my head up while prone, hold a rattle if put i my hand, put my hands together, I coo and socially smile and know my mom and dad
2 months
47
I can sit for a second when propped on my hands, transfer object back and forth, smile at a mirror
6 months
48
I can sit with trunk support, roll from my belly to my back, play with a rattle and my mom and dad can soothe me, I laught
4 months
49
I can sit by myself, crawl and pull to stand, pick up shit with 3 finger grasp, bang blocks and make 2 syllable sounds like mama and dada
9 months
50
What are 6 Ps of congenital hypothyroidism?
Pot bellied, pallor, puffy face, protruding umbilicus, protruding tongue and poor brain devo
51
6 year presents with fever, body aches and yellow/grey vesicles on uvula, soft palate and tonsils. Complications include HA, neck pain/stiffness and seizures.
Coxsackie A and B | Herpangina
52
Patient with FTT and poor weight gain at 3 months. CXR with water bottle shaped heart, increased pulmonary blood flow. Apical displaced anteiror leaflet of tricuspid valve and sale like appearance of anterior leaflet What do you see on EKG? Cause?
Ebstein anomaly: apical displaced septal tricuspid leaflet and leaflet dysplasia. Get right sided congestive heart failure adn right atrial enlargement leading to RBB as well as right atrial hypertrophy prone to arrythmia like SVT and atrial flutter
53
Patient presents with large local reaction to yellow jacket sting. What does this mean for bee sting allergy?
Low risk for anaphylaxsis treat with NSAIDS and ice IgE mediated response
54
What anoamlies are associated with high imperforate anus?
fistula to GU tract or clocal anomaly and other VACTERL anomalies... unilateral renal agenesis, neurogenic bladder of vesicourethral reflux are common
55
Patient with elevated Cl, urine pH of 7.4 and metabolic acidosis with normal anion gap presenting with emesis and abdominal pain
Distal RTA See hyperchloremic hypoKalemic metabolic acidosis with alkaline urine and normal anion gap due to defect in proximal tuble bicarb reabsorption or distal tubule hydrogen ion secreation
56
What is pathophys of distal RTA?
Can't acidify urine thus it's alkaline (>5.5), even though there is severe acidemia (HCO3 <15) thus renal Na is wasted--> leads to hyperaldosterism with increased K loss in urine.
57
How do we treat distal RTA
with sodium bicarb to correct hypoK and ECF volume depletion
58
What is pathophys of type 2 proximal RTA
Reabsorption of bicarb in proximal tubule is impaired-- get systemic acidosis and inappropriately elevated urine pH. Acidosis not usually as severe as type 1 and bicarb often greater than 15
59
risks of a ketogenic diet?
dyslipedemia, delayed growth and kidney stones
60
Pregnant women recently exposed to varicella. Not sure if she's ever had chicken pox. What do we do?
VZIG but no vaccine Never vaccine in preggers and ALL women pregnant with contact to VZV (uncovered x5 mins) w/o evidence of prior infection or IZ should have IG w/i 72 hrs
61
Patients with cholestasis have decreased bile which leads to decreased absoprtion of long chain fatty acids. What do we do for infants with cholestasis?
Formula with medium chain FA which dont require lipase for breakdown/absorption
62
What is the cause of beta thal intermediate?
beta globin gene defect ie poor translation of beta thal mRNA. It reduces production of beta globulin. Can have moderate anemia worse with illness/infection
63
How does patient with isoproanol intox present?
NS depression, signs of inebriation NO anion gap MA and they have fruity smelling breath. From hand sanitizer/antifreeze or rubbing alcohol
64
MCC of bactermia in kids?
Strep pneumo is most common but much less now bc of vaccine | Staph aureus and e.coli close seconds.
65
What embryonic layer gives rise to nervous system and peripheral nervous system? What happens if there is disruption at 3-6 weeks of devo?
Ectoderm | You get neurulation at this time, failure here can lead to neural tube defects
66
Abnormal _____ leads to disorders like spina bifida
caudal fusion (posterior neuropore)
67
Abnormal ___ leads to abnormalities like encephalocele or anecephaly
rostral fusion
68
Object permanence is a skill developed at ___
10 months
69
Infants at ____ can find a partially hidden object | At ____ they will look to floor when object dropped
7mo | 8mo
70
Infant that sits with trunk support, plays with rattle, stops crying at sound of parents voice and laughs out loud
4 months
71
At ___ infant sits momentarily propped on hands, transfer object, stranger anxiety, removes cloth from their face, smiles in mirror
6 monts
72
At ___ child climbs on furniture, bends to pick up toy, builds 3 cube tower, turns page in book, points to one body part, uses 3-5 words
15 months
73
What are predisposing factors for childhood schizophrenia? | When is prognosis worse?
MJ use and diagnosis of Autism or pervasive devo disorder. Prognosis worse in children and poor medication compliance. Negative symptoms respond less to meds
74
Occurs from repetitive motion injury to shoulder, have catching, locking or popping with history of glenohumearl dislocation
labral tear
75
Common in swimmers and other repetitive overhead activity and shoulder pain localized to deltoid and when laying on affected shoulder Dx and how to dx?
Rotator cuff tendinopathy Dx with US or MRI Tx ice, anti-inflammatories, PT
76
Patient with course facial features, HSP and recurrent upper respiratory infections, ivory colored papules on back, hydrocephalus, peripheral nerve entrapment. Normal conrea Dx? Genetics? Pathophys
Hunter Xlinked mucopolysaccharidosis; deficiency of iduronate sulfatase= accumulate heparn sulfate and dermatan sulfate live until adulthood
77
``` Infant course facial features, HSP, delays, corneal clouding recurrent URIs Dx? Genetics? Why? Survival ```
Hurler Autosomal recessive mutation on chrom 4 lysosomal hydrolase a-L-iduronidase deficiency = accumulate dermatan sulfate and heparan sulfate bc cant break it down Survival outcomes poor; devo cardiorespiratory failure and death by age 10
78
Pathophys of PSGN
Glomerular immune complexes from nephritognic strains of group A beta heymolytic strep (GAS) trigger complement activation + inflammation 1-3 weeks after pharyngitis or 3-6 weeks after cellulitis
79
Features of PSGN
Hematuria (tea colored), w or w/o red blood cell casts, variable proteinuria, edema, HTN LOW C3 and CH50 Good prognosis
80
Patient with dirty wound from cutting on metal fence. Has had 3 doses of tetanus vaccine, last one was 7 years ago. What is treatment?
High risk wounds (dirt, feces, soil, saliva) need Td or Tdap booster if >5 years since last one NO TIG needed since had at least 3 vaccines
81
What patients need tetanus immune globluin?
DIRTY wounds + less than three tetanus containing vaccines or unknown number
82
2yo child comes in with mom. Noticed erratic eye movements; move in all directions and has noticed episode where legs adn arms jerk sporadically. What is this concerning for?
Opsoclonus myoclonus syndrome = dancing eyes and dancing legs If <3yo, need to get screened for neuroblastoma as 40-50% with o/m under this age have neuroblastoma (but only 2-3% with neuroblastoma have this). Get urinary vanillymandelic acid level, MRI or US or MIBG scan
83
When are ASO levels useful?
Diagnosing strep infection in last 6 months; when suspicious of rheumatic fever, scarlet fever, acute post strep GN
84
Patient with recurrent infections with Staph, enteric gram negatives (salmonella, klebsiella, e coli) and fungi. Has history of subcutaneous and LN abscesses. Dx? Cause?
Chronic granulomatous disease X linked; neutrophil/macrophage cant activate respiratory burst to kill bacterial and intracell pathogens. Cant kill catalase + organisms, Serratia Have FTT, anemia, chronic diarrhea, HSP, hypergammaglobulinemia
85
Diagnosis of Chronic granulomatous disease
Dihydrohodamine 123 (DHR) oxidation test or nitroblue tetrazolium
86
Treatment for CGD
Lifelong PPx with antibiotics, antifungals and interferon gamma. May need BMT
87
Patients with recurrent bacterial infections with encapsulated organisms, recurrent skin infections, respiratory tract infections, angioedema. Type of infection?
Complement disorders | Screen with CH50 (antibody-dependent hemolytic assay)
88
What cell line is key for humoral immunity? What are made by these cells? What is their role?
Humoral = B cells B cells protect from infection; nuetrlaize toxins, facilitate opsonization, activate complement, direct NK cells and assist with aB-mediated cytotoxicity Make Ig G, M, A and E
89
____ present with recurrent otitis, sinus infections, PNA
B cell disorders (humoral immunity disorders)
90
Flow cytometry for lymphocyte phenotyping is useful for diagnosis of what diseases?
determines percentage of absolute number of T cell, B cell or NK cell subsets Diagnose SCID if complete absence Or agammaglobulinemia in isolated B cell deficiency
91
Pt eat abnormally large amounts of food in short period of time then induce vomitting, laxitives or excessive exercise. Often normal or overweight
bulimia nervosa
92
Pt overestimates body size, restricts calories and uses laxitives and vomits to rid self of calories when feel overeat
Anorexia; binge purge subtype
93
Patient overestimates body size, extreme caloric restriction and likely excessive exercise with body weight <85% expected
Anorexia; restrictive
94
How do we diagnose and tx eoe
scope and bx showing minimum 15 eosinophils per high powered field not resposive to reflux meds tx dietary modification and avoid triggers meds: swallowed fluticasone or budesonide may need mechanical dilation of esophagus
95
Cause of EoE
mixed immunoglobulin IgE and non IgE allergic response to foods and environment
96
Genetics of EoE
4:1 male to female predominance | 8% chance of offspring to have it if present in parent or sibling
97
MCC of unilateral abdominal mass in newborn period
hydronephrosis due to UPJ obstruction; leads to total blockage of flow. Dx made in newborn period or US and tx with surgery.
98
Newborn with absent radii and petichae all over. What else may be wrong?
TAR: thrombocytopenia with absent radius and NEVER have thumb anomalies rare genetics, also see cardiac defects; TOF, ASD and VSD
99
Tri-phalangeal thumb is seen in what two conditions?
Holt-Oram (CBC normal, family hx of heart adn limb defects from AD pattern of inheritance) Aase syndrome: patients with hypoplastic anemia
100
Progressive inability to bear weight on leg, painless limp and hip stiffness See flexion contracture of involved hip with slight external rotation. Pain with attempted internal rotation and abduction. imaging with femoral epiphysis flattened and fragmented with inferior and lateral femoral displacement
Legg Calve Perthe; impaired blood flow to femoral head--> avascular necrosis; insidious onset btwn 4-11 yo
101
Displacement of femoral neck through epiphyseal plate. Present with painful limp andLE is held externally rotated. Overweight child. Dx, what do you see on imaging and what is treatement
SCFE; more in males and more often unilateral with some increase risk in those with connective tissue disorder See widened and radiolucent physis to frank displacement of femoral head on proximal femur posteriorly and inferiorly Tx is KEY; PIN fixation
102
Physiology of developmental dysplasia of hip. | What do you see on PE and imaging
Femoral head with abnormal relationship to acetabulum; frank dislocation, partial or subluxation asymmetric skin folds of thigh/butt, LE of affected side may look shorter and limited ABduction if truly dislocated. US before 3 months; shows femoral head located laterally and superior to normal with shallow acetabulum.
103
Child with fractures starting around 12 mo, specifically long bones, blue sclera and worminan bones. Dx? Other associated findings?
OI type I (milder) Auto Dominant Type I collagen disorder, fx devo when child starts to become mobile (type II way worse and often fatal before 1st year of life). See sensineural deafness and subdurals
104
Heart defect with 3/6 holosystolic murmur at LLSB around day 3-4 of life.
VSD | will become symptomatic at 6-8 weeks of life if large enough once pulmonary vascular resistance drops
105
Patient with systolic murmur at LUSB and fixed S2 split
ASD
106
Patient with single S2, cyanotic and hypotensive at birth and tachynpnic
hypoplastic LHS
107
Patient with bloody stool, hematuria and tachycardic and periorbital edema. Labs with elevated BUN and Cr adn Pl of 90 with HgB of 7
HUS: hemolytic uremic syndrome; E.coli 0157:H7 or Shiga toxin
108
Osteochondritis of capitellum; about 7-12 yo and have pain in lateral elbow with decreased range of motion in extension
Panner disease
109
Pain with throwing around shoulder
Little League shoulder | proximal humeral epphysitis
110
How is Lithium excreated? What are contraindications to use?
inhibits neurotransmission and inhibits excitatory transmission NOT metabolized and excreted solely via kidney Chronic use--> lead to nephrogenic DI thus cant concentrate urine
111
What is paraphimosis and how is it treated?
complication of phimosis and that is retracted foreskin in uncirc'd penis that doesn't go back down. Needs prompt reduction; squeeze the glans then pul foreskin down; may need to place dorsal slit.
112
Bacterial or funtal infection of glans without foreskin infection
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