Peds Flashcards
1
Q
What is the most sensitive measure of intravascular volume status in children?
A
Heart Rate
2
Q
How does the compression to ventilation ratio change when delivering CPR to an infant or adolescent when there are 2 rescuers present vs 1 rescuer?
A
1 rescuer: 30:2
2 rescuers: 15:2
3
Q
At what age can the ‘adult’ setting be used on the AED during CPR?
A
> 8
4
Q
What is the most sensitive measure of adequate circulation in children?
A
Cap refill
5
Q
What are the first and second methods of treating hemodynamically stable Ventricular Tachycardia?
A
1) Amiodarone or procainamide for hypomagnesemia or hypokalemia.
2) Synchronized cardioversion up to 2J/kg
6
Q
What is the treatment for ventricular fibrillation or pulseless VT?
A
1) Nonsynchronized cardioversion up to 2J/kg followed by CPR
7
Q
What are the aspects of the secondary assessment in emergency managemnet (hint: SAMPLE).
A
S - SSx A - Allergies M - Meds P - Past MHx L - last meal E - events leading up to
8
Q
What is the indication and method of action for the following drug in pediatric cardiorespiratory resuscitation - Adenosine?
A
I: SVT
A: Causes AV block, interrupting re-entry circuits involving AV node
9
Q
What is the indication and method of action for the following drug in pediatric cardiorespiratory resuscitation - Atropine?
A
I: Bradycardia and AV block
A: Parasympatholytic drug - increase HR and blocks vagal stimulation
10
Q
What is the indication and method of action for the following drug in pediatric cardiorespiratory resuscitation - Sodium Bicarb?
A
I: Refractory metabolic acidosis, hyperkalemia
A: raises pH
11
Q
What is the indication and method of action for the following drug in pediatric cardiorespiratory resuscitation - Elemental Calcium?
A
I: Hypocalcemia, Hyperkalemia, Hypermagnesemia
A: Increases myocardial contractility and excitability
12
Q
What is the indication and method of action for the following drug in pediatric cardiorespiratory resuscitation - Dextrose?
A
I: Hypoglycemia
A: Increases BS
13
Q
What is the indication and method of action for the following drug in pediatric cardiorespiratory resuscitation - Epinephrine?
A
I: Asystole, bradycardia, pulseless VT/VF, shock
A: Increases SVR, Increases chronotropy and inotropy and thus CO.
14
Q
What is the indication and method of action for the following drug in pediatric cardiorespiratory resuscitation - Lidocaine?
A
I: Pulseless VT/VF, VT with pulse
A: Supresses ventricular arrhythmias
15
Q
What is the indication and method of action for the following drug in pediatric cardiorespiratory resuscitation - Amiodarone?
A
I: refractory SVT, and ventricular arrhythmias
A: Blocks ion channels and slows conduction through heart (widens QRS)
16
Q
What is the indication and method of action for the following drug in pediatric cardiorespiratory resuscitation - Procainamide?
A
I: SVT, atrial flutter, VT with pulse
A: Decreases conduction velocity throughout heart
17
Q
What two factors determine CO, what two determine BP?
A
CO = Stroke vol x HR BP = CO x SVR
18
Q
What drugs are commonly used for treatment of VT (4)?
A
Amiodarone, Procainamide, Lidocaine, Epinephrine
19
Q
What drugs are commonly used to treat SVT (3)
A
Adenosine, Amiodarone, Procainamide
20
Q
Provide the labs that are required for work-up and the antidote/treatment for the following substance overdoses - Acetaminophen
A
Labs: acetaminophen level, transaminases, PT
Tx: Oral N-acetylcysteine. Gastric emptying within 1 hr, activated charcoal within 4hrs.
21
Q
Provide the labs that are required for work-up and the antidote/treatment for the following substance overdoses - Anticholinergic Agents
A
Labs: Drugs screen
Tx: Physostigmine. Gastric emptying, activated charcoal, bowel irrigation. Benzo for irritation.
22
Q
Provide the labs that are required for work-up and the antidote/treatment for the following substance overdoses - Carbon Monoxide
A
Labs: Blood CO, urine dipstick (myoglobinuria), EKG
Tx: Oxygen
23
Q
Provide the labs that are required for work-up and the antidote/treatment for the following substance overdoses - Ethanol
A
Labs: serum ethanol
Tx: Supportive care, glc as needed
24
Q
Provide the labs that are required for work-up and the antidote/treatment for the following substance overdoses - Iron
A
Labs: Serum iron
Tx: Deferoxamine chelation. Gastric lavage, bowel irrigation, dialysis if severe
25
Provide the labs that are required for work-up and the antidote/treatment for the following substance overdoses - Methanol
Labs: Serum methanol, osmolal gap
Tx: Ethanol to block metabolism, sodium bicarb for metabolic acidosis, dialysis if severe.
26
Provide the labs that are required for work-up and the antidote/treatment for the following substance overdoses - Organophosphates
Labs: Blood screen
Tx: atropine sulfate, pralidoxime chloride, gastric lavage or activated charcoal
27
What blood lead level is required for the label "lead poisoning"?
> 20micrograms/dL
28
What are the clinical manifestations of chronic lead poisoning (4)? What is the serious complication?
1) developmental delay, behavioral problems, attention disorders, poor school performance.
2) Acute encephalopathy - high ICP, vomiting, ataxia, seizures
29
What is the treatment for symptomatic lead poisoning (3)?
1) remove from environment
2) Treat with chelation agents - EDTA, BAL, DMSA
3) Treatment must occur in lead free environment
30
What are the two requirements that must be met before graduating to a front-facing car seat?
1) >20lbs
| 2) >1 yr old
31
What age range is most likely to suffer from foreign body aspiration?
6-30 months.
32
What is the are affected, the clinical appearance, and the prognosis for first-degree burns?
affected - epidermis only
clinically - red, dry, tender, no blister
prognosis - heal within 1 week, no scarring
33
What is the are affected, the clinical appearance, and the prognosis for second-degree burns?
affected - dermis (superficial or deep)
clinically - superficial: painful, blister, wheeping. Deep: may or may not be painful
prognosis - superficial: resolve in weeks, no scar. Deep: may require graft, will scar.
34
What is the are affected, the clinical appearance, and the prognosis for third-degree burns?
affected - subcutaneous tissue
clinically - nontender
prognosis - require graft
35
What is the appropriate treatment for minor burns (superficial, less than 10% BA). What is the appropriate treatment for more serious burns?
Silver sulfadiazine if minor. Grafting if major.
36
What are three pathognomonic clinical signs of shaken baby syndrome?
1) intracranial hemorrhage
2) Widespread retinal hemorrhage
3) diffuse axonal injury
37
Provide child factors (5), maternal factures (5), and environmental factures (2) that are risk factors for SIDS
Child - male, low BW, premature, multiple gestation, black
Maternal - smoking, young, low SES, high parity, single parent
Environmental - soft bedding, obstructive objects in bed
38
Provide normal heart rate ranges for children: under 1 month, 1-3 months, 2-24 months, 2 - 10yrs, and 11-18 years old.
```
Less than 1 month: 80-160
1-3 mo: 80-200
2-24 mo: 70-120
2-10 yrs: 60-90
11-18 yrs: 40-90
```
39
Provide 5 conditions in which cyanosis will be evident sooner
1) high HgB (polycythemia)
2) decrease pH
3) increased CO2
4) increased T
5) increased ration adult HgB
40
What BP sign might indicate coarctation of the aorta or a ductal-dependent systemic blood flow with restrictive ductus.
Systolic BP >10mmHG in upper extremities
41
Describe how a hyperoxia test differentiates between cardiac and pulmonary anomaly
If PaO2 responds to 100% oxygen, then cardiac problem is unlikely. If non-responsive, then cardiac is likely.
42
What treatment should be started when CHD is suspected in the newborn?
PGE1 to keep ductus patent.
43
Provide the 3 cyanotic congenital heart lesions classified as ductal-independent mixing lesions
1) Truncus arteriosus
2) D-transposition of the great vessels
3) Total anomalous pulmonary venous congestion
44
Provide the defects present (2), clinical picture, anticipated heart sounds and murmurs (3), findings on exam (2), and treatment for truncus arteriosus
defects - single arterial vessel providing coronary, systemic, and pulmonic blood flow. VSD present.
Clinical picture - CHF in weeks
HSandM - systolic ejection murmur, loud ejection click, single S2
Exam - biventricular hypertrophy, increase pulmonary vascularity
Tx - surgical repair in neonatal period
45
Provide the defects present (3), clinical picture (2), anticipated heart sounds and murmurs (2), findings on exam (2), and treatment for transposition of the great arteries (3)
defects - aorta and pulmonary artery arise from the wrong ventricles, large ASD present, PDA present and required
Clinical picture - Cyanosis present at birth, tachypnea
HSandM - loud single S2, may have systolic murmur if VSD or pulmonic stenosis present
Exam - RVH, increased pulmonary vascularity
Tx - aterial anuloplasty, PGE1, arterial switch procedure in week 1.
46
Provide the defects present (2), clinical picture (2), anticipated heart sounds and murmurs (2), findings on exam (2), and treatment for total anomalous pulmonary venous connection (1)
defects - pulmonary veins drain into RA, R-L shunt achieved via PFO or PDA
Clinical picture - mild cyanosis, progressive HF
HSandM - wide and fixed S2, systolic murmur at P location
Exam - Cardiomegaly, increased pulmonary vascularity
Tx - elective repair prior to CHF
47
Provide the 3 cyanotic congenital heart lesions that are classified as ductal-dependent for pulmonary blood (L to R)
1) Tricuspid atresia
2) Tetralogy of Fallot
3) Ebstein Anomaly
48
Provide the defects present (4), clinical picture (2), anticipated heart sounds and murmurs (2), findings on exam (2), and treatment for tricuspid atresia w/ NRGA(1)
defects - Tricuspid atresia, PFO/ASD required, 90% have VSD, L-R shunt through PDA
Clinical picture - progressive cyanosis, tachypnea
HSandM - holosystolic murmur (VSD), continuous murmur of a PDA.
Exam - LVH, RAE
Tx - PGE1, hemi-Fontan (SVC to PA), Fontan at 2-4yrs (IVC and hepatic to PA).
49
Provide the defects present (4), clinical picture (2), anticipated heart sounds and murmurs (1), findings on exam (2), and treatment for tetralogy of fallot(1)
defects - VSD, pulmonary stenosis, large aorta, RVH
Clinical picture - cyanosis and tachypnea (tet spell)
HSandM - Pulmonary ejection click
Exam - LVH, decreased pulmonary vasculature
Tx - surgery at 3-6 months old.
50
Provide the defects present (2), clinical picture (2), anticipated heart sounds and murmurs (2), findings on exam (3), and treatment for Ebstein anomaly(1)
defects - low-riding tricuspid valve, hypoplastic RV
Clinical picture - cyanosis, CHF within days
HSandM - fixed split S2, systolic murmur (tricuspid regurg)
Exam - RAE, cardiomegaly, decreased pulmonary vasculature
Tx - heart transplant if severe. Surgery avoided.
51
Provide the 2 congenital heart lesions that are ductal-dependent for systemic blood flow (R to L).
1) Hypoplastic left heart syndrome
2) Interrupted aortic arch
bonus: Tricuspid atresia w/ TGA
52
Provide the defects present (3), clinical picture (1), anticipated heart sounds and murmurs (2), findings on exam (3), and treatment for Hypoplastic left heart syndrome(1)
defects - hypoplastic LV, mitral valve, ascending aorta. ASD. systemic blood flow through PDA.
Clinical picture - shock as PDA closes
HSandM - single S2, continuous murmur of PDA
Exam - Cardiomegaly, RVH, Pulmonary edema
Tx - PGE1, palliative surgery
53
Provide the defects present (1), clinical picture (1), anticipated heart sounds and murmurs (1), findings on exam (0), and treatment for Hypoplastic left heart syndrome(2)
```
defects - extreme coartation of aortic arch
Clinical picture - shock as PDA closes
HSandM - Continuous murmur of PDA
Exam - None
Tx - PGE1, anastomosis of coarctation
```
54
Provide the 4 acyanotic congenital heart defects that increase pulmonary blood flow
1) ASD
2) VSD
3) Common AV canal
4) PDA
55
Provide the 2 acyanotic congenital heart defects that result in pulmonary venous hypertension.
1) coarctation of the aorta
| 2) Aortic valve stenosis
56
Provide the defects present (1), clinical picture (2), anticipated heart sounds and murmurs (3), findings on exam (2), and treatment for ASD(2)
defects - L-R shunt through ASD
Clinical picture - asymptomatic, may have exercise intolerance
HSandM - fixed S2, systolic murmur (increased pulmonary flow), diastolic murmur (increased flow across tricuspid)
Exam - Cardiomegaly, increased pulmonary vascularity, RVH
Tx - Spontaneous closure in 1 yr, or surgery
57
Provide the defects present (2), clinical picture (1), anticipated heart sounds and murmurs (2), findings on exam (3), and treatment for VSD(2)
defects - L-R shunt through VSD
Clinical picture - CHF if defect large
HSandM - Small defects have high systolic murmur, wide S2 split (more pulmonary blood flow)
Exam - Cardiomegaly, LVH and LAE, possible RVH
Tx - Spontaneous closure if muscular, large ones corrected surgically
58
Provide the defects present (2), clinical picture (1), anticipated heart sounds and murmurs (3), findings on exam (2), and treatment for common AV canal defect(1)
defects - incomplete - ASD. Complete - ASD and VSD
Clinical picture - CHF in complete CAAV
HSandM - incomplete - mitral regurg. Complete - holosystolic, wide S2 split (more pulmonary blood flow)
Exam - Cardiomegaly, increased pulmonary vasc
Tx - repair at 6mo if large VSD to prevent CHF
59
Provide the defects present (1), clinical picture (2), anticipated heart sounds and murmurs (1), findings on exam (3), and treatment for PDA(2)
defects - L-R shunt through PDA
Clinical picture - pre-term, CHF if large
HSandM - holosystolic murmur of PDA
Exam - Cardiomegaly, increased pulmonary vasc, LAE/LVH/possible RVH
Tx - Indomethacin, coil embolism
60
Provide the defects present (1), clinical picture (2), anticipated heart sounds and murmurs (1), findings on exam (2), and treatment for CoA(2)
defects - High afterload
Clinical picture - ductal dependent systemic flow if severe, shock
HSandM - systolic ejection murmur (flow through narrowed artery)
Exam - Cardiomegaly, RVH/LVH
Tx - PGE1, surgical repair (anastomosis, dilatation)
61
Provide the defects present (1), clinical picture (2), anticipated heart sounds and murmurs (1), findings on exam (2), and treatment for Aortic Stenosis(2)
defects - Bicuspid valve
Clinical picture - ductal dependent systemic flow if severe, shock
HSandM - systolic ejection murmur (flow through narrowed artery)
Exam - Cardiomegaly, LVH, ST depression and T wave inversion consistent with ischemia
Tx - PGE1, surgical repair
62
What heart defect is associated with rheumatic fever
Mitral stenosis
63
When is Abx prophylaxis needed for dental surgery when concerned with the potential of Bacterial endocarditis (5)?
1) prosthetic cardiac valve
2) Previous endocarditis
3) Prosthetic cardiac material (within 6mo of surgery)
4) residual cardiac defect following corrective surg
5) Cardiac transplant with valve disease
64
What are the most common organisms that cause bacterial endocarditis in children?
GAS, S. aureus
65
Describe the common viruses that cause myocarditis (3), the unique clinical findings (4), and the treatment for patients (1)
1) adenovirus, coxsackie, echovirus
2) ST and T wave changes, CHF, low voltage, pericardial effusion and poorly working ventricles on ECHO
3) Supportive
66
Describe the mechanism of HOCM, the unique clinical findings (4), and the treatment for HOCM (3)
1) Defect left outflow due to thick septum
2) syncope, murmur of mitral regurg, S3, LVH
3) B-blocker, CCB, no sports.
67
Provide the 2 most common bradyarrhythmias that occur in children
1) Sinus node disfunction
| 2) Conduction block
68
Provide the 2 most common tachycardias that occur in children
1) Narrow QRS
| 2) Wide QRS
69
Provide the 2 most common sources of premature beats in children
1) Atrial
| 2) Ventricular
70
What is the mechanism of first-degree heart block and what are the EKG findings
1) Slowed conduction through the AV node
| 2) Prolonged PR interval, otherwise it is sinus rhythm with normal QRS
71
What is the mechanism for second-degree heart block? Contrast Mobitz type 1 and Mobitz type 2
1) Episodic interruption of the AV nodal conduction
2) Mobitz 1 (Wenckebach) - progressive PR elongation leading to a dropped beat.
3) Mobitz 2 - abrupt failure of conduction system below AV node, no progressive PR elongation, more serious as it can lead to complete heart block
72
What is fixed ratio AV block? What category of heart block does it fit into?
1) fixed ratio of P-wave to QRS waves (2:1, 3:1). Results from injury to AV node or Bundle. Serious as it can result in complete heart block
2) Type of second-degree heart block
73
What is significant about third degree heart block?
No conduction from atria to ventricles.
74
What is the appropriate treatment for Mobitz 2, fixed-ration AV block, and third degree heart block?
Pacemaker
75
Differentiate orthodromic reentrant circuits from antidromic reentrant circuits
1) orthodromic - reentrant circuit travels down AV fibers and up accessory pathway. Results in narrow complex tachy.
2) Antidromic - reentrant circuit travels down accessory pathway and up AV bundle. Wide complex QRS
76
What are the non-medical and medical therapies for narrow complex SVTs? How does treatment change if the patient has WPW?
1) non-med: vagal maneuver, RF ablation, cardioversion if unstable
2) Med: Adenosine useful to block conduction through AV node for SVTs caused by reentry
3) Maintenance Digoxin used. B-blocker used preferentially in WPW as digoxin slows AV conduction and favours antidromic WPW reentry.
77
Outline the treatment approach for wide complex tachycardia when the patient is: 1) stable, 2)unstable and in VF or pulseless VT, 3)In asystole
1) stable: IV Amiodarone, cardiovert if unsuccessful
2) unstable (VF, pVT): CPR, cardiovert
3) Asystole: give epinephrine until shockable rhythm present
78
What developmental parameter is the best measure of future intellectual potential?
Language
79
Provide a gross motor, fine motor, language, and social milestone for a 1mo infant
GM - raises head in prone position
FM - follow with eyes to midline only
L - startles to sound
S - fixes on faces
80
Provide a gross motor, fine motor, language, and social milestone for a 2mo infant
GM - raises head and chest in prone position
FM - follow with eyes 180 degrees
L - coos
S - recognizes parent
81
Provide a gross motor, fine motor, language, and social milestone for a 4mo infant
GM - lifts onto elbows, rolls front to back
FM - reaches for objects with both hands
L - laughs and squeal
S - initiates social interaction
82
Provide a gross motor, fine motor, language, and social milestone for a 6mo infant
GM - sits with support, rolls in both directions
FM - reaches for objects with one hand, transfers across midline
L - Babbles
S - recognizes objects or people as unfamiliar
83
Provide a gross motor, fine motor, language, and social milestone for a 9mo infant
GM - sits without support, pulls to stand
FM - pincer grasp, finger feeds
L - Speech sounds (mama, dada)
S - stranger anxiety, plays games (patty-cake)
84
Provide a gross motor, fine motor, language, and social milestone for a 12mo infant
GM - stands alone, a few steps
FM - voluntarily releases objects
L - 1-4 words, follows command with gesture
S - imitates, comes when called
85
Provide a gross motor, fine motor, language, and social milestone for a 15mo old child
GM - walks independently
FM - two-block tower, underhand throw
L - 4-6 words, follows one-step command
S - uses cup
86
Provide a gross motor, fine motor, language, and social milestone for a 18mo old child
GM - runs, walks up stairs with hand held
FM - three-block tower, uses spoon
L - 10-25 words, communicates needs with words
S - plays near but not with other children
87
Provide a gross motor, fine motor, language, and social milestone for a 24mo old child
GM - walks up and down stairs, throws overhand
FM - 4-6 block tower, uses spoon and fork, copies a line
L - 50+ words, two and three word phrases. 50% intelligible to stranger
S - removes simple clothing, parallel play
88
Provide a gross motor, fine motor, language, and social milestone for a 3yo child
GM - tricycle
FM - copies circle
L - 5-8 word sentences, 75% intelligible to stranger
S - knows age and gener, group play, shares
89
Provide a gross motor, fine motor, language, and social milestone for a 4yo child
GM - balance on one foot
FM - copies a cross
L - tells stories, 100% intelligible to stranger
S - dresses self, washes hands
90
Provide a gross motor, fine motor, language, and social milestone for a 5yo child
GM - skips on alternating feet
FM - draws person with six body parts
L - asks meaning of words
S - names four colours, understands 'rules'
91
Provide a gross motor, fine motor, language, and social milestone for a 6yo child
GM - rides bike
FM - writes name
L - identify written letters and numbers
S - knows all colours, knows R and L
92
What are three requirements of the diagnosis for ADHD?
1) present before 7
2) Present >6mo
3) Present in two environments (ie. home and school)
93
What are three characteristic features of ADHD?
Inattention, hyperactivity, impulsivity
94
Provide two stimulant drugs used in the treatment of ADHD and their mechanism of action. What are common side effects (2)?
Methylphenidate and dextroamphetamine. Increase dopamine and norepinephrine in the CNS. Can result in insomnia and anorexia.
95
What is a non-stimulant used in the treatment of ADHD? What is its mechanism?
1) Atomoxetine
| 2) SNRI
96
What are three characteristic impairments of autism spectrum disorder?
1) Social interactions
2) Communication
3) Behaviour
97
Provide two features that differentiate Asperger's from Autism
1) Patient does not have significant language development problem (however, does not understand metaphors/sarcasm and non-verbal behaviours)
2) Wishes to form friendships but cannot
98
What is the cause, SSx, and treatment for Hand-foot-and-mouth disease?
1) Coxsackie A virus
2) Vesicular rash on hands, feet, and oral mucosa with erythema
3) Supportive treatment
99
What is the cause, presentation, and treatment for chickenpox? How does treatment change in the immunocompromised child?
1) VZV
2) Red papules that become vesicles and rupture. Pruritic and begin on trunk.
3) Tx: supportive with antipyretic and bathing. If immunocompromised give VZV IG.
100
What is the cause presentation, and treatment (3) of molluscum contagiosum?
1) Poxvirus
2) flesh-coloured umbilicated papules spread via auto-innoculation.
3) Cryotherapy, cantharidin, imiquimod cream
101
What is the cause presentation, and treatment (3) of Verrucae (warts)?
1) HPV
2) asymptomatic
3) liquid nitrogen, cantharidin, imiquimod cream
102
What is the cause presentation, and treatment (3) of Pityriasis Rosea?
1) Unknown
2) Asymptomatic, may be pruritic. Starts with a herald patch followed by smaller lesions on trunk in christmas tree pattern
3) Supportive. Antihistamine or topical steroid if pruritic.
103
What are the causative agents, presentation, and treatment of bullous impetigo?
1) S. Aureus
2) bullae on errythematous base
3) 1st gen cephalosporin (Cephalexin) or Septra
104
What are the causative agents, presentation, and treatment of non-bullous impetigo?
1) GAS, S. Aureus
2) vesicles -> pustule -> rupture with crust (honey coloured)
3) 1st gen cephalosporin (Cephalexin) or Septra
105
What are the causative agents, presentation, and treatment of diaper rash?
1) Candida
2) papules, pustules, and scales with satellite lesions
3) antifungal cream (nystatin)
106
What are four possible treatments for acne vulgaris?
1) Benzoyl peroxide - decrease P. acnes colonization
2) Topical retinoids - anticomedogenic (need to use sunscreen too)
3) topical Abx (clinda and erythromycin) - prevent P. acnes
4) Systemic Abx (tetracycline, doxycycline, erythomycin)
107
Provide 4 characteristics of psoriasis
1) papules becoming plaques with scales
2) extensor surfaces
3) Onycholysis
4) non-pruritic
108
What are 2 treatments for psoriasis?
1) topical steroids
| 2) Methotrexate or biologics (etanercept)
109
What are the causative agents of errythema multiforme (3)? What is the treatment?
1) HSV1
2) Adenovirus
3) EBV
Tx - supportive with oral antihistamines and moist compress
110
What is the presentation of SJS, what causes it, and how is it treated?
1) errythematous and purpuric macules and bullae
2) drug hypersensitivity reaction - NSAIDs, penicillins, anti-epileptic drugs
3) barrier isolation and fluid support
111
What is the presentation of TEN, what causes it, and how is it treated?
1) full detachment of epidermis
2) drug hypersensitivity reaction
3) treat as if large second degree burn
112
What can be expected on blood and urine analysis is a suspected DKA? Provide some clinical signs (4).
1) blood - BG high, pH low, PaCO2 low. Low Ca, K, Phos
2) Urine - glucose and ketones
3) Clinically - polyuria, polydipsia, fatigue, emesis
113
What are the signs of hypoglycemia? Differentiate those caused by catecholamine (4) release from those due to cerebral glucopenia (4)
1) Cat - shakes, sweaty, flushing, tachycardia
| 2) Cer - sleepy, confused, mood change, seizure/coma
114
What are some common causes of diabetes Insipidus (4)? What can be expected when examining blood and urine concentrations (3)? How is this treated?
1) head trauma, CNS infection, tumor, recent head surgery (craniopharyngioma removal)
2) Dilute urine (less than 300 mOsm), low-specific gravity (loss of free water), hemoconcentration (>295 mOsm)
3) DDAVP, an ADH analogue
115
What are are the causes of SIADH (4)? What is expected in analysis of the urine and blood (3)? How is it treated (2)?
1) encephalitis, brain tumor, head trauma, psychiatric
2) High urine osmo (>200), high urine Na (>20), low blood osmo (less than 280).
3) Tx - fluid restriction, hypertonic fluid to raise Na loss
116
Provide the primary cause of hyperthyroidism in children, the clinical picture (5), and the appropriate treatment (2)
1) Graves disease (high free T4)
2) high apetite, tachycardia, diarrhea, jittery, heat intolerance
3) PTU or methimazole
117
Describe the clinical picture for hypothyroidism (4), differentiate primary from secondary hypothyroidism, and provide the treatment.
1) cold intolerance, low apetite, constipation, lethargy
2) Primary - high TSH. Secondary - low, normal TSH. Total T4 diminished in both
3) levothyroxine
118
What hormones are deficient and which exist in excess in congenital adrenal hyperplasia? What can be seen clinically in male and female infants? How is this treated?
1) deficient in aldosterone and cortisol due to lack of 21-hydroxylase. Excess 17-hydroxyprogesterone and androgen production.
2) Clinical - salt wasting dehydration, low energy due to hypoglycemia. Females - ambiguous genitalia.
3) exogenous cortisol and Aldosterone, surgery for females.
119
Describe the clinical picture in Addison disease (4). What is the cause? What is the treatment for Addisonian crisis? What lab values are typically abnormal in an addisonian crisis?
1) weakness, N/V, emotional liability, salt craving, tanned joints due to high ACTH
2) Primary adrenal insufficiency
3) Fluid corrections and stress dose of glucocorticoids
4) low BG, low Na, high K
120
What are two common causes of cushing disease in the peds population? What diagnostic test is used to confirm the presence of cushing syndrome?
1) Adrenal tumors or microadenoma of pituitary (high ACTH)
| 2) Dexamethasone suppression test
121
What test can be used to differentiate gonadotropin-dependent precocious puberty from gonadotrpin-independant precocious puberty?
GnRH administration. Gonadotropins increase if cause is GDPP
122
How can hypoparathyroidism and vitamin D deficiency as a cause of hypocalcemia be differentiated based on lab values?
1) low PTH -> low Ca, high Phos. As PTH impact on kidneys to secrete Phos is diminished.
2) Low Vit D -> low Ca, and low Phos. PTH high, thus high kidney excretion of Phos.
123
What is the formula for maintenance fluids in a child? What electrolyte balance must be present? What is typically given in practice?
1) 4ml/kg first 10kg, 2ml/kg next 10kg, 1ml/kg each additional kg.
2) 3mEq Na and 2 mEq K for each 100 cc fluids
3) Typically 1/4NS with 5% dextrose and 20mEq/l KCl
124
Comment on heart rate, urine osmol, BUN, and blood pH in a mild (less than 5%) dehydrated child.
HR elevated, urine osmo >600, BUN normal, blood pH normal
125
Comment on HR, mucous membranes, fontanelle, tearing, mental status, urine osmo, BUN, and blood pH in a moderately dehydrated child (5-10%)
HR elevated, mucous membranes dry, fontanelle depressed, tearing absent, mental status altered, urine osmo >800, BUN elevated, blood pH mildly acidotic
126
Comment on HR, BP, cap refill, mucous membranes, fontanelle, tearing, mental status, urine osmo, BUN, and blood pH in a severely dehydrated child (>10%)
HR greatly elevated, BP depressed, capp refill >3sec, fontanelle sunken, tearing absent, mental status depressed, urine osmo maximal, BUN high, blood pH profound acidosis
127
What is the first step in managing severe dehydration (ie hypovolemic shock)? How does this differ from mild-moderate dehydration?
1) IV bolus of isotonic fluid at 20ml/kg
| 2) Mild to moderate dehyration can be replaced orally
128
What are 3 causes of hyponatremia? What are the clinical signs (4)? How is it treated?
1) dehydration, SIADH, adrenal insufficiency
2) confusion, lethargy, decreased DTR, seizure
3) If due to excess water, then fluid restrict. If due to dehydration, then provide fluid resus. Hypertonic saline avoided as it can damage pontine.
129
What is the risk of rapid sodium correction in a hypernatremic child?
Cerebral edema
130
Provide 5 causes of hyperkalemia. What signs appear on EKG (3). How is it treated (4)?
1) acidosis, dehydration, K-sparring diuretics, excessive infusion, renal failure
2) Wide QRS, T-wave spikes, ST depression
3) Calcium gluconate, insulin/bicarb, hyperventilate, kayexylate
131
What are 3 causes of hypokalemia? How is it corrected?
1) DKA, alkalosis, loop diuretics
| 2) correct pH, replenish stores
132
What is the anion gap formula?
Na - (Cl- + HCO3-), range: 16 (+ or -4).
133
What is the normal PaCO2 in children? What is the expected response of PaCO2 in a metabolic acidosis (provide formula)
1) 35-45
| 2) PaCO2 = 1.5HCO3 + 8 (+ or -2)
134
How is chronic abdominal pain defined?
3 bouts severe enough to affect activities in 3 month period.
135
How soon does the appendix perforate after inflammation begins? What is seen during investigations (CBC, xray, CT)? What is given if the appendix perforates?
1) 36 hours
2) high WBC with left shift, xray may show fecalith, CT shows inflammation
3) IV Abx - amp, gent, metronidazole
136
Where is the most common location for intussusception to occur? What may act as lead points (3)? What are some clinical findings (3)? How is it treated (2)?
1) ileo-colic
2) meckel's diverticulum, intestinal polyp, lymphoma
3) rectal bleed (currant jelly), palpable tubular mass, colicky episodes
4) Contrast or air enema. Laparotomy with reduction.
137
What electrolyte distrubance is observed in the patient with severe emesis?
hypochloremic hypokalemic metabolic alkalosis
138
Describe the clinical picture in pyloric stenosis (3). What are the risk factors (2)? What is the treatment (2)?
1) hypochloremic hypokalemic metabolic acidosis, projectile non-bilious vomiting, papable mascular non-tender epigastric mass
2) male, errythromycin
3) NG tube and correct of electrolyte imbalance, pylorotomy
139
Describe the clinical picture in malrotation with volvulus (3). What are the risk factors (1)? What is the treatment (1)?
1) bilious emesis, gas in stomach on xray, lactic acidosis if bowel becomes ischemic
2) Improper rotation of bowel inutero
3) Surgery
140
Describe the clinical picture of GERD (5). What is involved in the work-up (2)? What are some lifestyle modifications that can improve symptoms (3)? What are the medical therapies available?
1) vomiting, poor weight gain, dysphagia, substernal pain, esophagitis
2) pH monitoring, manometry
3) left-sided sleeping if >12mo, milk-thickening agents, avoid trigger foods
4) PPI > H2 RA>antacids
141
How is salmonella as a cause of acute gastroenteritis treated depending on age range?
1) If less than 3 months and febrile, admit for blood culture and IV Abx. Do LP and UA
2) If >3 months and febrile, admit and start IV Abx if blood culture positive
142
What Abx are used in the treatment of C Diff (2)?
Metronidazole and vancomycin
143
What are the radiographic findings of Hirschsprung disease (2)? What are the clinical findings (3)? How is it diagnosed? What is the treatment?
1) distended proximal bowel, no gas or feces in rectum
2) disease generally limited to distal rectosigmoid colon, palpable stool on exam with no stool in rectum, no meconium in 24-48 hrs
3) Rectal biopsy (absence of ganglion cells)
4) Surgical removal of aganlionic portion
144
Where is the meckel diverticulum found in the GI tract? How is it diagnosed? What are the most common clinic symptoms (3)?
1) Small-intestine, 100 cm from the ileo-cecal valve
2) Meckel scan - radio-isotope for ectopic gastric mucosa
3) painless rectal bleeding > volvulus > diverticulitis
145
Comment of the following features are they present in Crohn Disease (never, rare, sometimes, common): Malaise, wt loss, rectal bleed, adbominal mass, perianal disease, ileal involvement, stricture, fistula, skip lesions, transmural inflammation, granulomas, long term cancer risk.
Malaise-c, wt loss-c, rectal bleed-s, adbominal mass-c, perianal disease-c, ileal involvement-c, stricture-s, fistula-s, skip lesions-c, transmural inflammation-c, granulomas-s, long term cancer risk-increased.
146
Comment of the following features are they present in Ulcerative colitis (never, rare, sometimes, common): Malaise, wt loss, rectal bleed, adbominal mass, perianal disease, ileal involvement, stricture, fistula, skip lesions, transmural inflammation, granulomas, long term cancer risk.
Malaise-s, wt loss-s, rectal bleed-c, adbominal mass-r, perianal disease-r, ileal involvement-r, stricture-n, fistula-n, skip lesions-r, transmural inflammation-n, granulomas-n, long term cancer risk- greatly increased.
147
Provide 5 therapies used in the treatment of IBD
1) 5-ASA
2) Abx
3) Corticosteroids
4) Methotrexate
5) Colectomy in UC
148
What are the teratogenic effects of isotretinoin, diethylstilbestrol, streptomycin, and valproate?
iso - facial and ear anamolies, CHD
DES - vaginal adenocarcinoma during adolescence
Strep - deafness
Valproate - spina bifida
149
Provide examples of autosomal dominant genetic diseases (5)
1) Achondroplasia
2) Adult polycystic kidney disease
3) Hereditary spherocytosis
4) Marfan syndrome
5) Neurofibromatosis
150
Provide examples of autosomal recessive genetic diseases (6)
1) Congenital adrenal hyperplasia
2) cystic fibrosis
3) Infantile polycystic kidney
4) PKU
5) sickle cell disease
6) Tay-Sachs
151
Provide clinical features of Trisomy 21 (8)
flat occiput, flat facial profile, upslanted palpebral fissures, small ears, flat nasal bridge, small mouth with large tongue, singular palmar crease, clinodactyly, sandal sign
152
Provide clinical features of Trisomy 18 (8)
prominent occiput, narrow forehead, low-set ears, micrognathia, CHD, short sternum, clenched hands with overlapping figures, rocker-bottom feet, death within 1 year
153
Provide clinical features of Trisomy 13 (8)
Microcephaly, microphthalmia, cleft lip and palate, CHD, omphalocele, clenched hands with overlapping fingers, polydactyly, cryptorchidism, death within 1 yr.
154
Provide clinical features of Turner syndrome (6). What is done for mosaic patients who are 45XO/46XY
1) shield-chest, hypoplastic nipples, webbed neck, low hairline, short stature, lack of pubertal development
2) Gonadectomy
155
What are the clinical features of Klinefelter syndrome (XXY) (4). When do they become obvious?
1) female body habitus, gynecomastia, small phallus and testes, infertility
2) Not noted fully until puberty
156
What is the mechanism that causes prader-willi syndrome? Provide some clinical features (5).
1) Uniparental maternal disomy or deletion of paternal C15
| 2) short stature, hypogonadotrophic hypogonadism, small genitals, hypotonia, uncontrollable apetite
157
What is the mechanism that causes angelman syndrome? Provide some clinical features (5).
1) Uniparental paternal disomy or deletion of maternal C15
| 2) maxillary hypoplasia, short stature, ataxic gait, tiptoe walk, laughter, mental retardation
158
What type of genetic disorder is Fragile X? Provide some clinical features (4).
1) Trinucleotide repeat disorder
| 2) macrosomia, macroorchidism, mental retardation, large jaw and ears
159
What are the clinical manifestation of galactosemia (3)? What are found on lab exam? What is the treatment?
1) accumulation of galactose-1-phosphate in liver, kidneys, and brain leads to: liver failure, renal dysfunction, emesis
2) Labs: disrupted LFTs, hypoglycemia, and aminoaciduria
3) Remove galactose from diet
160
What are the clinical signs of PKU (4)? What is the treatment?
1) mental retardation, hypertonia, tremors, light complection
2) Dietary phenylalinine restriction
161
What type of genetic disorder is Ornithine Transcarbamylase Deficiency? What is elevated in the blood? What are some signs (2)? How is it treated?
1) X-linked
2) Hyperammonemia (due to urea cycle defect)
3) lethargic and seizures within 24-48 hrs of protein feeds
4) Low protein diet, exploitation of alternative pathways for nitrogen excretion
162
Provide the formula for the adjusted reticulocyte count and comment on its meaning.
1) ARC = (measure Hct/expected Hct) * retic count
| 2) If less than 2, then insufficient erythropeitic response
163
Provide 5 types of microcytic anemias
1) Iron deficiency
2) Thalassemia
3) Sideroblastic
4) Lead poisoning
5) Anemia of chronic disease*
164
Provide 2 types of macrocytic anemias
1) Vit B12 def
| 2) Folate def
165
How is mild to moderate iron deficiency anemia treated?
3-6mg/kg/day of elemental iron. Therapy is continue for 8 weeks following return of normal hgb levels.
166
Discuss homozygous a-thalassemia in terms of genetic deletions, hgb composition, clinical signs, and treatment
1) 4 a-globin genes deleted (Bart disease)
2) HgB Bart (gamma tetramers)
3) death in utero
4) Tx - N/A
167
Discuss hemoglobin H (a-thalassemia) in terms of genetic deletions, hgb composition, clinical signs, and treatment
1) 3 a-globin gene deleted (Hgb H disease)
2) Hgb H 30-40% (beta tetramers)
3) N/A
4) Tx - RBC transfussion every 3-5 weeks, chelating agents with desferrioxamine
168
Discuss a-thalassemia minor in terms of genetic deletions, hgb composition, clinical signs, and treatment
1) 2 a-globin gene deletion
2) Normal HgB composition (normal electrophoresis)
3) Anemic
4) Tx - none
169
Discuss homozygous B-thalassemia in terms of genetic deletions, hgb composition, clinical signs, and treatment
1) 2 b-globin gene deletion
2) Reduction or abscence of HgB A, presence of HgB F
3) Extramedullary hematopoiesis with MSK deformities
4) Tx - transfussions every 3-5 weeks with RBCs, chelating agents (desferrioxamine)
170
Discuss B-thalassemia minor in terms of genetic deletions, hgb composition, clinical signs, and treatment
1) 1 b-globin gene deletion
2) Reduction of HgB A, presence of HgB F
3) Anemia
4) Tx - none
171
Autoimmune hemolytic anemia can be caused by IgG and IgM. Comment on the difference in these two mechanism by commenting on their ability to activate complement, their classification as 'warm' or 'cold' reactive, the result of the combs test, and the location of the hemolysis
IgG - warm reactive, does not fully active compliment, combs positive, opsonized extravascularly
IgM - cold reactive, activates complement, combs negative, intravascular lysis.
172
With respect to hereditary spherocytosis, provide its inheritence pattern, clinical signs (5), and treatment (2)
1) AD
2) If severe - hemolytic anemia, growth failure, splenomegaly, transfusion requirement in aplastic crisis, hyperbilirubinemia with gallstones
3) Dx with osmotic fragility test. Tx - transfusions if aplastic crisis, splenectomy after 6yrs old.
173
With respect to sickle cell disease, provide its mechanism of inheritence, the findings on electrophoresis, clinical manifestations (5), and appropriate therapy (4)
1) AR
2) HbS on electrophoresis, absent HgA. Both B-globin genes knocked out
3) Clinical: splenomegaly, delayed sexual maturation, gallstone formation, vaso-occlusive crises (priaprism, dactylitis, AVN femoral head), stroke
4) transfusion if stroke or priaprism. Hydroxyurea for maintenance. Splenectomy. Penicillin prohphylaxis for splenic infarcts.
174
With respect to G6PD, comment on its inheritence, precipitating factors in causing a crisis, appearance on blood smear (2), clinical findings during crisis (2), and appropriate treatment (2).
1) X-link recessive
2) oxidative stress on RBCs (sulfonamides, nitrofuratoin)
3) smear - Heinz bodies, blister cells
4) clinical - jaundice, dark urine
5) RBC transfusion if severe crisis, fluids, alkalinization of urine
175
What can cause a B12 deficiency (3)? What are the clinical signs (4)? What is seen on blood smear (3)? How is it treated (1)?
1) Dietary deficiency, pernicious anemia (lack of intrinsic factor), bacterial intestinal overgrowth
2) glossitis, weight loss, neurologic sequelae, vitiligo
3) Hypersegmented neutrophils, basophilic stippling, Howell-Jolly bodies
4) IM vit B12
176
What causes folate deficiency (2)? What on the clinical presentation separates it from B12 deficiency? What is the appropriate treatment?
1) Dietary deficiency (goats, evaporated, heat-treated milks)
2) No neurologic sequelae
3) 1mg folate orally
177
Describe Diamond-Blackfan syndrome. What is seen on electrophoresis? What is seen clinically (4)? What is the treatment (3)?
1) congenital pure red cell aplasia
2) HgB F increase
3) short stature, web neck, cleft lip, triphalangeal thumb
4) steoirds, transfussion, bone marrow transplant
178
What is Fanconi Anemia? What is seen clinically (4)? What is seen on smear? How is it treated?
1) AR disorder resulting in pancytopenia
2) cafe au lait spots, microcephaly, short stature, no thumbs
3) HgB F increase
4) Bone marrow transplant
179
What is the mechanism that causes ITP? When does it typically present? What is the appropriate treatment based on severity(3)?
1) Antiplatelet antibodies bind to platelet and they are destroyed in the RES
2) Weeks following a viral illness
3) mild - most resolve within 6 months. Moderate - if plts low, give steroids and IVIG and antiD. Severe - IVIG or splenectomy
180
What is the clinical picture of DIC (3)? What is it classified as? What is the treatment?
1) bleeding at venipuncture sites, GI blled, hematuria
2) Microangiopathic disease
3) Supportive
181
What factors are deficient in Hemophilia A and B? What are appropriate treatments for both? What lab value is abnormal?
1) A is deficient in VIII, B is deficient in IX
2) Factor replacement. DDAVP can be used in HemA as it increases VIII (monitor for hyponatremia)
3) aPTT
182
What are the clinical manifestations of von Willebrand disease (3)? What is the appropriate treatment (2)?
1) bleeding - mucous membranes, epistaxis, menorrhagia
| 2) Tx - DDAVP which stimulate release of vWF or attenuated vWF concentrate.
183
What coagulation factors are vitamin K dependent? What cause vit K deficiency (3)? What labs are abnormal? What is the treatment?
1) 2, 7, 9, 10
2) malabsorption (CF), maternal warfarin or anti-convulsant use, refusal of vit K prohpylaxis
3) PT and aPTT
4) parenteral vit K
184
What coagulation factors are found in cryoprecipitate (3)?
8, 13, and fibrinogen
185
Describe the impact of severe combines immunodeficiency disease (SCID) (2). What is the treatment?
1) deficits in humoral and cell-mediated immunity, patients susceptible to wide range of infections in first months of life
2) bone marrow and cord blood tansplant
186
Differentiate oligoarticular JIA, RF+ polyarticular JIA, and RF- polyarticular JIA based on lab values and clinical presentation.
1) Oligoarticular: less than 5 joints, large joints, anterior uveitis, ANA positive
2) RF+ polyarticular: >5 joints, small joints, symmetric distribution, rheumatic nodules present, erosive
3) RF- polyarticular: >5 joints, small and large joint involvement, better prognosis, anterior uveitis
187
What are significant lab test fidnings in SLE? How is SLE treated?
1) ESR elevated, combs positive, C3 low, ANA positive
| 2) NSAIDs for MSK issues, immunosuppression if renal or CNS involvement
188
What is the clinical presentation of HSP? What is the mechanism? What is the treatment?
1) nonthrombocytopenic pupura, colicky GI pain, glomerulonephritis, lower extremity edema
2) IgA immune complex deposition in vessel walls
3) Supportive. NSAIDs for MSK pain. Corticosteroids for GI and renal involvement
189
What are the diagnostic criteria for Kawasaki disease (6)? What is the biggest concern? What is the treatment (2)?
1) Warm cream - fever for 5 days, conjunctivities, rash edema, adenopathy (unilateral), mucosal involvement (erythema, crusting).
2) coronary artery aneurysm
3) IVIG, aspiring, corticosteroids.
