Pediatrics Flashcards
1
Q
What is the most common upper airway disease in children?
A
Croup
2
Q
What is the most common age group affected by croup?
A
Age 3 mos- 5yrs
3
Q
What is the most likely dx for a 1 yr old pt presenting in the winter with rhinorrhea, sore throat, hoarseness, tachypnes, a barky cough and inspitatory stridor?
A
Croup
4
Q
What is the most common organism that causes croup?
A
Parainfluenza virus (enveloped ss RNA)
Note: influenza, RSV can also cause croup
5
Q
What is the next step in management for a pt suspected of having croup?
A
Neck XR ( if not sure -steeple sign)
1)Humidified Oxygen
2)Nebulized Epinephrine
and Steroids
6
Q
What is the most likely diagnosis in a child (age between 3-7yrs) who presents with drooling, high fevers, sniffing/tripod position, muffled voice, dysphagia, inspiratory stridor, toxic appearing?
A
Epiglottitis
Also hv high suspicion in pt who did not complete immunizations or are from foreign country
7
Q
What is an important next step in management for a pt with epiglottitis?
A
Transfer to OR (or hospital if in clinic)
ENT/Anesthesia consult
Secure Airway
Antibiotics (Ceftriaxone) + Steroids
Rifampin to household contacts if H. influenzae positive
8
Q
What are some common organisms associated with Epiglottitis?
A
Strep pyogenes Strep pneumoniae Staph aureus Mycoplasma H. influenza (not as common d/t vaccines)
9
Q
Once a pt. with epiglottitis is stabilized (ABC), what are the next steps in management?
A
Neck XRAY (thumb print sign)
Blood cultures
Laryngoscopy/ Nasopharyngoscopy (to visualize Cherry Red Epiglottis) Do this in OR
Remember: DX work up only takes place after pt is intubated.
10
Q
What is the most likely dx in a 2y/o pt with a brassy cough, high fever, respiratory distress all following recent URI?
A
Bacterial Tracheitis
11
Q
What is the most common organism associated with Bacterial tracheitis?
A
S. aureus
12
Q
What age group is commonly affected by Bacterial tracheitis?
A
Children
13
Q
What is the next step in management for a child suspected of having bacterial tracheitis?
A
If pt is in need of airway–> Intubate
If pt breathing is stable --> CXR (subglottic narrowing and ragged tracheal air column) This is a clinical dx but can do Laryngoscopy Blood cultures Throat cultures
14
Q
What is the treatment for bacterial tracheitis?
A
Dicloxacillin (PO)
Oxicillin, Nafcillin (IV)
15
Q
How can epiglottitis and croup be distinguished. clinically?
A
Croup: Barky Cough
Epiglottitis: No cough
16
Q
What age is most common for foreign body aspiration?
A
Children
17
Q
What is the next step in management for a child who presents with sudden onset choking, gagging, coughing, possible wheezing, drooling, overall respiratory distress while playing with small, smooth, toys and unilateral decreased breath sounds ?
A
First step: CXR (hyperinflation d/t air trapping, lack of mediastinal shift downward in decubitus position)
Rigid Bronchoscopy to visualize and remove object
18
Q
What anatomic sites are most common area of foreign body aspiration?
A
> 1 yr: Larynx
19
Q
If a pt suspected of having a foreign body aspirated and presents with wheezing, what should NOT be given?
A
DO NOT give Bronchodilators (b/c this canallow object to move further down into airway)
20
Q
What is the best next step in management of a 6 mos old child presenting in January with wheezing, rhinorrhea, low-grade fever, cough, tachypnea, and prolonged expiration, intercostal retractions on exam?
A
CXR (hyperinflation; patchy atelectasis associated with bronchiolitis)
21
Q
What is the most accurate test for dx’ing bronchiolitis?
A
Viral Ag Test: Nasopharyngeal Swab
this test is not necessary for diagnosis. Its dx’d clinically
22
Q
What is the standard treatment for bronchiolitis?
A
Supportive care
Hospitalize If severe (tachypnes >60b/min) and try nebulized B2 agonist
23
Q
What are some ways to prevent RSV?
A
Breastfeeding (IgA)
RSV IVIG or palivizumab (anti-RSV F protein Ab)
24
Q
Who should receive RSV prophylaxis?
A
Preterm babies
Those with bronchopulmonary dysplasia
25
What is the age group that most commonly get viral pneumonia?
Age
26
What are common organisms associated with viral pneumonias?
RSV
Parainfluenza virus
Adenovirus
27
What age is common for presentation of bacterial pneumonia in children?
>5y/o
28
What organisms are comonly associated with bacterial pneumonia in children?
S.pneumo
Mycoplasma
Chlamydia pneumoniae
29
What is distinct between viral and bacterial pneumonias on presentation?
Viral: URI BEFORE the pneumonia
Bacterial: acute onset high chills/fever, decreased breath sounds
30
What are some typical features associated with Chlamydia trachomatis pneumonia?
```
Stacatto cough
Absence of fever/wheezing (unlike RSV)
Eosinophilia
Infants (1-3 mos)
+/- Congenital at birth
```
31
What is the best way to distinguish Chlamydia pneumoniae from Mycoplasma pneumonia clinically?
It cannot be done.
| Note: chlamydia pneumonia pneumonia is very different from that associated with chalmydia trachomatis
32
What is the best way to confirm Mycoplasma pneumoniae infection?
IgM titers against mycoplasma
33
If there are pneumatoceles (cavitations) present on CXR, what is the most likely organism?
S. aureus
34
What is the best initial test in dx'ing a child with possible pneumonia?
CXR
| other tests include: CBC w/ diff, viral/mycoplasma IgM titers, Blood Cultures
35
What are the best treatment options for bacterial pneumonias?
Outpt: Amoxicillin (alt, cefuroxamine or amox/clavulanate)
Inpt: IV cefuroxamine
Chlamydia/Mycoplasma: Macrolide
36
What chromosome and common gene are affected in cystic fibrosis?
```
Chromosome 7
CFTR gene (codes for Cl- channel)
```
37
What is the best initial test to dx CF?
Sweat Chloride test (x2 on separate days showing sweat Cl- >60mEq/L)
38
What are some common associated findings associated with Cystic Fibrosis?(9)
```
Meconium Ileus (new born)
Recurrent respiratory infection/cough (bronchopulmonary aspergillosis)
FTT
Malabsorption (fatty stools, Fat sol Vit deficiency)
Rectal Prolapse (infants)
Nasal Polyps
Male infertility
Pancreatic Exocrine Insufficiency
```
39
What is the best initial test for a new born who failed to pass meconium in 48 hrs after birth?
Abdominal Xray (see microcolon)
40
What are two common organisms that infect the airway of pt with CF?
S. aureus
Pseudomonas
H. influenzae
41
At what age should PFT's be initiated in pt with Cystic Fibrosis?
5 or 6 yrs of age
(PFT's show an obstructive pattern initially (FEV1/FVC
42
What type of testing should all CF pts receive to guide therapy?
Genotyping -to determine presence of G551D mutation. If present can give Ivacaftor (VX-770) to restore CFTR function
43
What tests are used to monitor disease pregression?
CXR
| PFTs
44
What treatments are shown to improve survival?
Ibuprofen
| Azythromycin and other Ab's during exacerbations
45
What Ab's are used to treat CF -related infections?
Mild Disease: Macrolide, TMP/SMX, or Cipro
Documented S.aureus or Pseudomonas: Piperacillin+ Tobramycin or Ceftazidime
Resistant Pathogen: Inhaled Tobramycin
46
What immunizations should CF pts receive?
All immunizations plus pneumococcal and annual flu shot.
47
What is the most common cause of death in infants between 1-12 mos of age?
Sudden Infant Death Syndrome (SIDS)
48
What is the best test to detect apnea in an infant?
Polysomnography
49
What is the most common cause of inspiratory stridor in infants within first 2 ws -6mos of life?
Laryngomalacia
50
How is laryngomalacia diagnosed?
Clinical dx but can do Laryngoscopy to confirm (to see degree of stenosis)
51
What Laryngeal pathology is associated with meningomyelocele, hydrocephalus, and Arnold Chiari malformation?
Vocal Cord Paralysis
52
What is the best test to dx ?
Flexible Bronchoscopy
53
What are some common signs of CHF in infants?
```
FEEDING problems:
difficulty feeding
sweating while feeding
easily fatigued (during feeding)
FTT/poor wt gain
```
54
What is the best initial test in working up a child with possible Congenital Heart Disease?
CXR (enlarged heart)
and
EKG (initially, LV hypertrophy)
55
What is the most accurate test for dx'ing CHD/CHF in a child
Echocardiography
56
What is the most common Congenital Heart Defect?
VSD
57
What type of murmur is associated with VSD?
Holosystolic +/- thrill (dependent upon size of the defect)
Note: smaller defects are more audible than larger ones.
58
What is a complication of VSD?
Eisenmenger Syndrome
59
What is the definitive treatment for Eisenmenger Syndrome?
Hear-Lung transplant
60
What is treatment for CHF in child?
Medications initially until Surgery is possible
61
What is a complication for any high flow lesion?
Endocarditis
62
What is the most common type of ASD?
Scundum type (area of foramen ovale)
63
What type of heart sounds/murmur is associated with ASD?
WIDE FIXED SPLIT S2 (increased flow through PV-->closes later)
Systolic Ejection Murmur @ L upper sternal border (flow through PV)
64
What is the treatment for a patent ASD?
Surgery if it does not close spontaneously by age 4 or 5yrs old
65
What chromosomal abnormality is commonly associated with Endocardial cushions?
Trisomy 21 (Down Syndrome)
66
What is the murmur associated with Endocardial Cushions?
Loud systolic murmur with thrill
| Diastolic flow murmur
67
What is the mumur associated with Patent ductus arteriosus?
Continuous machine-like (to and fro)
68
What physical exam findings can be associated with PDA?
Wide Pulse pressure (Systolic-diastolic)
| Bounding Pulses
69
What is the treatment for pt with PDA?
Preterm: Indomethacin (NSAID)
Term: Surgical Closure
70
What abnormality can be seen on CXR and EKG in a pt with Pulmonary Artery Stenosis?
```
When severe CXR:
Enlarged Rt Hrt
Decreased Pulm vascularity
Pulm Artery Dilation
Darker (blacker) lung fields (b/c less flow)
```
EKG: Right Ventricular Hypertrophy
71
What murmur is associated with Pulmonic Stenosis?
Short, low-pitched with systolic ejection Click
72
What skeletal finding can be present in Aortic Stenosis adult type in a child?
Rib Knotching (d/t collateral circulation over time)
73
What is the treatment for Coarctation of the Aorta infantile type?
Infant/Neonate: Prostaglandin E1 infusion (keep DA patent) and surgery when stable
In older, treat HTN first then surgery
(Surgery is definitive)
74
What is the most common cyanotic congenital heart condition presenting beyond infancy?
Tetrology of Fallot
75
What are the associated anomalies of Tetrology of Fallot?
Pulmonary Stenosis (infundibulum just below valve)
Overriding Aorta
Right Vent Hypertrophy
VSD
76
What is a common finding associated with TOF?
Tet Spells (hypercyanotic attacks)
Increased resistance to blood flow to the lungs at the infundibulum
77
What might a child do to relieve a tet spell?
Flex knees/hip to chest of infant
Child can Squat
(increase SVR and reverse the shunt to increase pulmonary flow)
78
What heart sound/murmur are associated with TOF?
Single S2 and Harsh
79
What are some typical CXR findings associated with TOF?
CXR: Boot-shaped heart (d/t upslanting APEX) with blackened lung fields
80
How is TOF treated?
Oxygen
BBlockers
PGE1 (cyanotic at birth)
Surgery 4-12 mos
81
What is the most common cyanotic heart condition presenting in newborn period?
Transposition of Great Vessels (TPGV)
82
What metabolic disorder tends to be associated with infants dx's with TPGV?
Maternal Diabetes
83
What heart sound can be associated with TPGV?
Loud, single S2
84
How is TPGV treated?
PGE1 to maintain patent Ductus Arteriosus
Surgery ASAP
85
When should prophylactic Ab's be given to prevent Endocarditis?
Only prophylax for dental procedures iff:
Prosthetic Valves
Un-repaired/Persistent Congenital Heart Disease
H/o Endocarditis
Cardiac transplant with Valve problems
86
At what age should routine BP checks begin?
3yo (must check in all 4 extremities)
87
What cause of HTN should always be on differential for a pediatric pt?
Renal causes
88
Who should be worked up for secondary HTN?
Newborn: think renal thrombosis
Child: coarctation, renal parenchyma, endocrine, meds
Adolescent: Obesity, Renal-->Urinary
89
What tests should be ordered initially to evaluate a pediatric pt for HTN?
```
CBC
Urinalysis/Cx
BUN/Cr
Electrolytes
Glc
Calcium
Uric Acid
Lipid Panel (Essential HTN and Family Hx)
```
90
What additional tests can be done to evaluate for specific etiologies of HTN?
Echocardiogram
Renal U/s
VoidingCystourethrogram
(if recurrent UTI
91
What is the standard of care to manage HTN in pediatric pt?
Obese: Lifestyle modifications
If Lifestyle modifications dont work--> Medications
92
What is the best initial medicinal therapy in pediatric pt with HTN?q
Diuretics or B-blockers
| Can add Calcium Channel blockers if needed
93
What medication should be added if renal involvement?
ACE inhibitors
94
What is the most common cause of diarrhea in infancy?
Rotavirus (ds DNA)
95
What is the most common cause of bloody diarrhea?
```
Campylobacter (G- motile, flagella)
Amoeba (e. histolytica)
Shigella (G- rod, no spores)
Salmonella (G- rod, motile)
E. Coli (O157:H7, G- rod)
C.diff (G+, spore-forming)
Giardia (anaerobic protozoan)
Cryptosporidium: Protozoan in immunocompromised
```
96
What is the best initial test for evaluating a pediatric pt with diarrhea?
Stool sample: cultures, wbc ct, blood, ouvum and parasites
| C.diff toxin if h/o Ab use
97
What is the best initial treatment for a pediatric pt with diarrhea?
Hydration
Replace Fluids/electrolytes
(Never use antidarrheals in peds pt)
98
With which organisms should Ab's be given for diarrhea in pediatric pt?
Shigella (TMP/SMX)
Campylobacter (Erythromycin if severe or dysentery )
Salmonells (
99
What is a complication that must be monitored for in pediatric pt with cryptosporidium-related diarrhea?
Malnutrition
100
What is the most common cause of Acute Renal Failure in young children?
Hemolytic Uremic Syndrome (HUS)
101
What organisms are associated with HUS?
E.coli O157:H7 (most common)
Shigella, Salmonella, Campy
102
What tests should be ordered in work up for pt suspected of having HUS?
```
CBC
Peripheral Smear
Urinalysis
Urine protein/cr ratio
Electrolytes
BUN/Cr
Coombs Test (neg)
```
103
What long term management should take place for pt who had/recovered from HUS?
BP monitoring for 5yrs
BUN/Cr monitoring for 2-3 yrs
104
What is next step ina pt suspected of having Giardiasis?
Duodenal Aspirate and Bx or Immunoassay
105
What is the best initial test in the workup for malabsorption?
Sudan Black stain (stool)
| Can also do serum trypsinogen screen to assess pancreatic problem
106
What is the best test to confirm Malabsorption in a pediatric pt?
72-hr stool collection for fecal fat
107
What is the best initial test for Protein malabsorption?
Spot stool Alpha-1-Antitrypsin level
| Note, cannot evaluate protein malabsorption directly
108
What additional tests should be done when managing malabsorption?
Vitamin/Mineral levels: Iron, Ca, Mg, Zn, VIt D, A, B12, folate
109
What conditions are Celiac disease pts at increased risk of developing?
Osteoporosis (decreased Ca)
GI malignancies (T-cell lymphoma)
110
What is the best initial test in dx'ing Celiac disease in a child?
Antitransglutaminase Ab
111
What is the most accurate test for dx'ing Celiac disease?
Bx with Histology
112
What is the next step in management for a child
H2 Blockers (first line medical therapy in children)
113
What is the best initial treatment for children who present with symptoms of GERD?
Feeding technique and consistency adjustments
114
What is the best initial test to dx GERD in a child?
Esophageal pH monitoring
115
What is the best initial test for a first-born Caucasian boy presenting with non-bilious vomiting, hypochloremic, metabolic alkalosis with palpable, firm, mobile mass in epigastric region?
Abdominal Ultrasound
| Note: Hypochloremic Metabolic Alkalosis is pathagnomonic for Pyloric Stenosis
116
What is the treatment fot Pyloric Stenosis?
Correct fluid/electrolytes first then
| Pyloromyotomy
117
What is a key distinguishing feature between Pyloric Stenosis and Duodenal Atresia?
Pyloric Stenosis: Non-bilious vomiting
Duodenal Atresia: Bilious vomiting
118
What condition should be suspected in a child presenting with small bowel obstruction WITHOUT a h/o prior surgery?
Volvulus
119
What should be suspected in an infant presenting with Bilious emesis with abdominal pain?
Malrotation or Volvulus
120
What is the best initial test when malrotation or volvulus suspected?
Ultrasound (see SMA and SMV inversion and duodenal obstruction)
or
Barium Enema
121
How is malrotation/volvulus treated?
Surgery
122
What is the next step in management for a male child about 2yo presenting with intermittent, PAINLESS bleeding per rectum with no other complaints of physical exam findings?
Tc-99 pentechnetate Scan (radionucleotide scan) to detect gastric mucosa in lower abdomen -->dx Meckel Diverticulum
123
What are some common features associated with Meckel Diverticulum?
```
Rule of 2's:
2:1 m:f
2 types of tissue (gastric, pancreatic)
2 in long
2 ft from ileocecal valve
2% of population
```
124
What is the embryonic origin of Meckel Diverticulum?
Omphalomesenteric Duct
125
T/F: Meckel Diverticulum is a pseudodiverticulum?
FALSE: Meckel Diverticulum is true, all 3 bowel wall layers involved (mucosa, submucosa, muscularis propria)
126
How is Meckel Diverticulum treated?
Surgery
127
What is a complication associated with Meckel Diverticulum
Spontaneous Intussusception (diverticulum acts as lead point)
128
What are typical features associated with the presentation of Intussusception?
Child
129
What is the best initial test in a pt presenting with episodes of colicky abdominal pain current jelly stools?
```
Abdominal Xray (r/o obstruction)
Air/Barium Enema (dx and rx intussusception)
```
[Manual/Surgery if enema does not reduce]
130
What is the most common age to develop UTI in pediactric population?
Boys= 1yo
| Girls>2yo (most girls will hv one UTI b/f age 5)
131
What is the best initial test to dx UTI?
Urinalysis
132
What is the most accurate test to dx UTI in pediatric population?
Urine Culture
133
What is the best initial treatment for uncomplicated UTI in child?
Amoxicillin or TMP/SMX
134
What is the best treatment for pyelonephritis in a child?
IV Ceftriaxone or
| Ampicillin + gentamicin
135
What age is contraindicated for use of sulfonamides and nitrofurantoin?
136
What age is contraindicated for the use of quinolones?
137
What is is contraindicated for the use of Tetracyclines?
138
What is the next step in management for a child treated for UTI?
Repeat Urine culture 1 wk after Ab stopped (confirm sterile urine) Recheck periodically for 1-2 yrs
139
What are the indications for a Voiding Cystourethrogram?and renal ultrasound in a child with UTI?
```
First febrile UTI w/ :
FHx of renal/urological disease
Poor growth
Hypertension
non-E.coli organism isolated from culture
```
All children with 2 or more FEBRILE UTIs
140
What is the next step in management for an ill-appearing 2yo pt presenting with high fever, vomiting and a h/o multiple UTIs since birth and no formal evaluation/followup?
Urinalysis/Cx
| VCUG and Renal U/s
141
If a child presenting with sx of urinary tract infection with h/o recurrent UTI since birth without formal evaluation or followup has a VCUG that shows abnormal urinary backflow, what is the most important intervention to prevent permanent renal damage?
Antibiotics for 1 yr from the time of diagnosis of any grade of Vesicoureteral Reflux (VUR)
Abs of choice: TMP/SMX or Nitrofurantoin (cannot use latter in infants decrease pyelonephritis-->decrease renal scarring-->decreases risk of reflux nephropathy]
142
When is surgery indicated for VUR?
UTI while on Ab prophylaxis
New scar develops
Ab's dont help
143
What is the most common presentation associated with Obstructive Uropathy in children?
Infection/Sepsis
144
What is the most common cause of bladder obstruction in boys and what are 2 key findings?
Posterior Urethral Valves
| walnut-shaped mass over pubic symphysis and weak urinary stream
145
What is the most common cause of palpable abdominal mass in Newborns?
Hydronephrosis and Polycystic Kidney Disease
146
What is the best initial test to dx Obstructive Uropathy?
VCUG and renal Ultrasound
147
What is the next step in management for a child (5-12 yo) presenting with a few days of dark-colored urine who had a URI 1-2 weeks before, who now has BP >150/90 and lower extremity swelling?
Urinalysis
ASO titer
Complement (esp C3)
148
What is the most accurate test for dx'ing PSGN (APGN)?
Anti-DNase Ag
149
What is the best initial treatment for a pt with PSGN (APGN)?
```
Antibiotics: Penicillin (Erythromycin only if allergic)
Supportive care (no antihypertensive meds in peds though!)
```
150
What are some common clinical features associated with Berger's Disease (IgA Nephropathy)?
```
Gross Hematuria
Recent URI/GI illness
Mild proteinemia
HTN
NORMAL C3 complement
```
151
What is the best test to dx Berger's Disease?
Renal Bx
152
What is the best treatment for Berger's Disease?
BP control/Supportive care
153
What is the most likely diagnosis in a young boy presenting with asymptomatic microscopic hematuria/intermittent gross hematuria after URI, FHx of renal problems and sensorineural hearing loss?
Alport Syndrome
154
What should be checked on physical exam in a pt suspected of having Alport Syndrome?
Eye exam for ocular abnormalities
155
What renal abnormality is suggested in a pt with bilateral flank masses and hypoplasia?
Autosomal recessive Polycystic Kidney Disease (infantile type)
156
What are some symptoms that should raise suspicion for PCKD (AR)?
```
Potter's Sequence
Hypertension
Oliguria
Acute renal failure
with prenatal h/o Olighydramnios
```
157
What is the best initial dx step for a pt suspected of having PCKD (AR)?
Ultrasound -Kidney and Liver
158
What is the treatment for PCKD (AR)
Dialysis and transplant
159
What is the most common form of persistent proteinuria in school-aged children and adolescents?
Orthostatic Proteinuria
160
What is the most common age range for a child to present with Nephrotic Syndrome?
between 2-6y/o
161
What is the next step in management for a 3yo child who presents with swollen eyes, urinalysis showing 3+proteinuria, serum albumin
Oral Steroids (Great response with Minimal Change Disease)
162
How long should steroid therapy be continued when treating Minimal Change Disease?
Daily 4-6wks then taper by alternating days for 2-3 months without bx.
163
What are the typical findings associated with Nephrotic Syndrome in pediatric pt?
Proteinuria (>40mg/m2/hr; Cr normal)
| Hypoalbuminemia (
164
What is the next step in management for a pt who has a relapse of symptoms while on steroids for minimal change disease?
Switch to
Cyclophosphamide, Cyclosporine, or
High-dose, pulsed methylprednisolone
165
What are possible complications in a child with Nephrotic Syndrome?
Infection (must get Pneumococcal and Varicella)
| Thromboembolism (loss of anticoag protein factors)
166
What are the features associated with a presentation of CAH, 21-hydroxylase deficiency?
```
Ambiguous genitalia (Masculinization of female genitalia)
Vomiting
Dehydration
Hyperkalemia
Hyponatremia
Hypoglycemia
```
167
What are the metabolic derrangements associated with CAH-21hydroxylase deficiency?
```
Decreased Progesterone--> Deoxycorticosterone:results in Decreased Aldosterone production
Low Aldosterone leads to:
Hyponatremia
Hyperkalemia
Dehydration
```
Decreased 17-hydroxyprogesterone-->11deoxycortisol: results in decreased Cortisol production
Low Cortisol leads to :
Increased ACTH release
Adrenal Hyperplasia
Hypoglycemia (decreased counter-regulation of insulin)
Increased Androgen Synthesis-->Ambiguous genitalia
168
What is the best initial test in dx'ing a pt with CAH?
Serum 17-Hydroxyprogesterone (elevated)
Can also test Cortisol (low), Renin (High),
Aldosterone (Low)
169
What is the best confirmatory test to dx pt with CAH?
17 Hydroxyprogesterone before and after IV bolus of ACTH
170
What is the best treatment for a pt with CAH?
Hydrocortisone (cortisol replacement)
Fludrocortisone (Aldosterone replacement)
Corrective surgery for female genitalia
Note: Must increase doses of replacement hormones in times of stress (ie, infection/pre-op)
171
What is the best next step in management for a 4yo child presenting with fever for 5 days, injected conjunctiva, red tongue, cracked lips, desquamating rash and edema on hands, and painful palpable lymph nodes in the neck?
IVIG and high-dose Aspirin (to prevent cardiac sequela)
Add Warfarin if platelets are very high
[CRASH and Burn = Kawasaki, a clinical dx is all that is needed to treat)
Then do other tests for work up and baseline status
172
What is a common, life threatening complication of Kawasaki disease?
Coronary abnormalities
173
What should the workup for Kawasaki diseaseinclude?
ESR
CRP (elevated at 4-8wks)
CBC (platelets, esp 2-3wks)
2D Echo/EKG for base line and repeat at 2-3wks and again at 6-8wks (ck for: EARLY- myocarditis/pericarditis , LATE-coronary aneurysm by wk 2-3)
174
What is the most likely dagnosis in a child b/w 2-8yo with Abdominal pain, palpable Purpura (maculopapular rash), Fever, and recent URI?
Henoch-Schonlein Purpura (HSP)
[small vessel vasculitis]
175
What immune components are key factors in HSP?
IgA and C3 (deposit in arterioles, capillaries, venules)
176
How are IgA Nephropathy (Berger's) and HSP distinguished?
Berger's mainly 20-30yo pt, renal only
| HSP mainly children; systemic disease (renal, skin, connective tissues, joints, and GI)
177
What medical conditions is HSP associated with?
Intussusception
Arthritis
Glomerularnephritis/nephrosis
178
What is the best way to diagnose HSP?
H&P (purpura on legs/bottom, fever, abdominal pain, child with recent URI)
179
What test can be done to support a dx of HSP?
```
CBC (wbc, platelets increased, anemia)
ESR
IgA, IgM (increased)
Anticardiolipin/Antiphospholipid Ab
Urinalysis (RBCs, WBCs, casts, albumin)
```
Note: Skin bx is definitive dx but not necessary to dx!
180
How is HSP treated?
If GI or Renal sx: Steroids
| If Anticardiolipin/Antiphospholipid Ab's + and/or thrombosis occurs: Aspirin (antiplatelet)
181
If a term infant presents with a Hb of 9-11 mg/dL with no signs of cyanosis or respiratory distress, what is the treatment?
NO Treatment. This is physiologic anemia and can last until about 3 months of age (due to EPO suppression at birth)
182
At what age would you expect to see a baby present with iron deficiency anemia?
9-24 mos
183
What is the treatment for Iron-def Anemia in pediatric pt?
Oral ferrous salts
Limit cow's milk
Continue Iron replacement for 8 wks after Blood levels normalize to replenish stores
184
What condition is most likely to be dx'd in a child presenting with hyperactivity, aggression, learning disability, in the context of impaired growth, constipation, and mental lethargy?
Lead Poisoning
185
What is the best initial test to screen for Pb toxicity?
In high-risk pt: Blood Lead levels at 12 and 24 months (not finger prick)
186
What is the maximum cut off for acceptable Blood Lead Level in a child?
5mcg/dL
187
What labs/results can be associated with Pb toxicity?
CBC: Microcytic Hypochromic Anemia
Free Erythrocyte Porphyrins (FEP)
Peripheral Smear: Basophilic Stippling
XRAY (long Bones): Dense Lead Lines
188
What is the next step in management whena child is found to have a blood lead level >15mcg/dL and >45mcg/dL?
>15mcg/dL: Report to Health Department
| >45mcg/dL: Chelation with (Calcium Disodium EDTA)
189
What are the most common causes of mortality in pediatric cases of Sickle Cell Disease?
Infection(sepsis) and Acute Chest Syndrome
| Acute Spelnic Sequestration
190
What organisma are pts with sickle cell disease particularly susceptible to?
Encapsulated organisms (S.pneumo, H. influenza, N. meningitidis) d/t autoinfarction (usually by age 5)
191
What is the best diagnostic test to dx and screen (newborn) for sickle cell disease?
Hb Electrophoresis
192
When is the earliest a pt can be screened for sickle cell disease?
Prenatally (if both parents have trait)
CVS: 10-12 wks
Amniocentesis: 14-18 wks
193
What is the indication for blood transfusion in pt with Sickle Cell disease?
Symptomatic Anemia (SOB, Chest pain)
194
What is the indication for Hydroxyurea in pt with Sickle Cell Disease?
Symptomatic Anemia
3+ crises/year
Life-threatening compliations (stroke, acute chest syn, splenic crisis)
195
What is the indication for Exchange transfusion in a pt. with Sickle Cell Disease?
Life-threatening complications
| Before high-risk surgery
196
What is the routine management for pts living with Sickle Cell Disease?
Penicillin Prophylaxis (start at 3mos continue to 5yo)
Immunizations: All plus Pneumococcus @ 2mos, meningococcal @2yrs, and Influenza @6mos then annually
Daily Folic Acid supplement
[Note: definitive ttreatment for sickle cell diseae is bone marrow transplant]
197
What is the best initial test in the work up of a pt suspected of Thalassemia?
Hb Electrophoresis (also most specific)
198
What additional tests should be included in dx'ing B-thalassemia?
CBC (low retic ct, microcytic, RBC with increased nucleated RBCs)
Peripheral Smear (Target cells)
Iron Studies (Ferritin, Transferrin-high)
Bilirubin(elevated indirect)
LDH(elevated)
Haptoglobin (low)
199
What Electrophoresis results ae expected in a pt with B-Thalassemia Major?
Increased HbF (Alpha tetramers d/t excess alpha globin chains)
Decreased/Absent HbA
Variable HbA2
200
What findings can be associated with B-thalassemia Major?
Anemia, hypersplenism, cardiac decompensation. expanded medullary space (esp face and skull), extramedullary hematopoeisis, hepatosplenomegaly
201
How should bacterial infections be managed in pt with sickle cell disease?
Aggressive Ab therapy
202
How is B-Thalassemia major treated?
Transfusion (to maintain Hb>9mg/dL)
Iron Chelation (Defuroxamine)
Vit C Supplementation
Splenectomy (d/t hypersplenism, but not until 5yo)
[Note: Bone marrow transplant will cure]
203
What is included in routine management for a pt living with B-Thalassemia Major?
Folic Acid Supplementation
Immunizations
Daily Penicillin for prophylaxis
Growth Hormone replacement (excess Iron -->GH deficiency)
204
What types of bleeding are associated with Platelet or Von Willebrand Factor(vWF) Deficiency/Dysfunction?
Mucosal bleeding [oropharynx, conjunctiva, epistaxis, vaginal-(menorrhagia)]
Skin (surface): Petechiae, small ecchymoses, purpura, ,
Post-op bleeding
205
What type of bleeding is associated with Clotting Factor Deficiencies?
Deep Bleeds: Hemarthrosis, bleed into muscle, Large Ecchymoses, Hematoma
206
For what components of platelet activation/aggregation is the glycoprotein IIb/IIIa receptor affinity increased upon platelet activation?
vWF and fibrinogen
207
What are the best initial test to work up a pediatric pt with a bleeding disorder?
CBC (platelets, low platelets most common cause in kids)
Bleeding Time
PT(Extrinsic)
PTT(Intrinsic)
208
What are the best confirmatory tests to work up bleeding disorders?
```
Mixing Studies (for factor deficiencies)
Clotting Factor Assays (when you know specific factor to test)
Quantitative vFW Ag/activity assay (Ristocetin but do vWF levels first, Ristocetin only tests activity)
```
209
How should a mixing study be interpreted that shows correction of prolongation when normal plasma added to pt?
Clotting Factor deficiency
210
How should a mixing study be interpreted that shows NO correction/partial correction of prolongation when normal plasma added to pt?
Inhibitor to clotting factor is present in pt sample
211
How should a mixing study be interpreted that shows increased prolongation with clinical bleeding when normal plasma added to pt?
Antibody against a clotting factor is present in pt. sample
212
How should a mixing study be interpreted that shows prolongation with a prolonged PTT and no bleeding when normal plasma added to pt?
Lupus Anticoagulant -->Predisposition to clotting (remember, the term "anticoagulant" is based on what the Ab does to coag labs (prolong.) It is actually a procoagulant in vivo!!)
213
What is the next step in management for a pt with a h/o hemophilia who has been well controlled suddenly develops bleeding diathesis?
Order Mixing Study (results should indicate Factor inhibitor present)
214
What is the treatment for a pt with Hemophilia A with minor bleeding?
DDAVP (desmopressin) +
Aminocaproic Acid or Tranexamic Acid
(the latter two are antifibrinolytics)
215
What is the treatment for a pt with Hemophilia A with major bleeding?
Factor VIII replacement
216
What is the treatment for a pt with Hemophilia B with minor bleeding?
Factor IX concentrates
217
What is the treatment for Hemophilia B with major bleeding?
Factor IX concentrates
Note: all bleeds in Hemophilia B receive Factor IX concentrates
218
What is the treatment for a pt with vWD subtype 1 with minor bleed?
DDAVP (desmopressin)
219
What is the treatment for a pt with vWD subtypes 2 and 3 with major bleeds?
plasma-derived vWF with Factor VIII concentrates
220
What is the most likely diagnosis in a child between 1-4 yo presenting with petechiae, purpura on her bottom, isolated thrombocytopenia,normal PTT, PT, and bleeding time who reports a recent URI?
Idopathic Thrombocytopenic Purpura (ITP)
221
What is the underlying mechanism causing ITP?
AutoAb against platelet surface proteins
222
What is the next step in dx'ing a child between 1-4 yo presenting with petechiae, purpura on her bottom, isolated thrombocytopenia,normal PTT, PT, and bleeding time who reports a recent URI?
CBC
Peripheral Smear (to r/o TTP/HUS, which would have schistocytes)
Bone Marrow Bx
Sonogram (to check spleen)
[If actively bleeding treat according to severity of bleed]
223
What is the treatment for ITP?
Steroids (in mild disease) immediately
IVIG (life-threatening bleed)
Chronic ITP: Rituximab or Splenectomy
224
What is the next best step in management for a child b/w the ages of 9mos-5yo who is reported to have had a generalized tonic-clonic seizure lasting 102) fever and family hx of simple febrile seizure?
Reassurance
Manage the fever
Evaluate for meningitis (physical exam)
225
What findings indicate an increased risk of epilepsy in a child with febrile seizure?
```
Atypical seizure (>15 min, >1/day, focal findings)
FHx of epilepsy and first seizure
```
226
What is a seizure disorder of infancy marked by clusters of mixed flexor/extensor spasms that persist for minutes with brief intervals between?
Down Syndrome (most common CNS disorder associated with West Syndrome (infantile Spasm)
227
What is the EEG finding associated with West Syndrome?
Hypsarrhythmia (very high voltage slow waves with spike and sharp wave irregularly interspersed)
228
What is the treatment for West Syndrome?
ACTH, prednisone, Vigabatrin, B6 (pyridoxine)
229
What is the firstline treatment for a child with Absence seizures?
Ethosuxamide
230
What is the first line treatment for Partial Seizures?
Carbamazepine or Valproic Acid
231
What is the definition of fever without a focus in children?
Child38/100.4
| Fever lasts 3wks)
232
What are the indications for Ab in a child with Fever without a focus?
Documented rectal temp>100.4/38
| WBC>15,000 with > 1500 Neutrophils +bands
233
What is the workup for Fever without a focus in a Neonate?
Hospitalize
Panculture (blood, urine)
Prohylactic Ab: cover GBS, Listeria, E.coli Ampicillin/Sulbactam or 2/3 gen Cefalosporin)
234
What is the most common organism associated with Fever without a focus in an infant?
Strep pneumo
235
What is the treatment for Fever without a focus in an infant?
Well-appearing: IM Ceftriaxone (single dose)
Toxic Appearing: Empiric IV Ab
236
What is the best next step in managing a baby who presents with poor feeding, irritability, lethargy, with development of recent vomiting and has had a fever of 101.3? On exam there are bulging fontanelles and left lateral gaze palsy. Immunizations are up to date.
Give empiric Ab: Vancomycin, Ceftriaxone or Cefoxatime (based on clinical suspicion for meningitis with Increased ICP)
(Note only add steroids if possibility of Hib-rlated meningitis with increased ICP)
237
What is the treatment for a child with Strep pneumo meningitis?
Penicillin or 3rd gen Cephalosporin for 10-14 dys
238
What is the treatment for a child with Gram -neg (E.coli) meningitis?
Third gen Cephalosporin for 3wks
239
What is the treatment for a child with empirically treated and no organism isolated meningitis?
Third gen Cephalosporin for 7-10 dys
240
What is the treatment for a child with HiB meningitis?
Ampicillin for 7-10 dys plus Dexamethasone
241
What is the treatment for a child with N. meningitidis meningitis?
Penicillin for 5-7 days
242
What is the prophylactic treatment for bacterial meningitis and who should receive it?
Rifampin for N.meningitidis and HiB to all close contacts
243
What is the most common complication of meningitis, esp with S. pneumo?
Hearing loss
244
What are some complications associated with meningitis especially if treatment is delayed?
Clots, Mental retardation, neurologic dysfunction
245
Seizure and persistent fever are complications of meningitis associated esp with which causal organism?
HiB
246
What complications are associated with meningococcal meningitis?
Waterhouse Freidrichsen Syndrome [Adrenal helorrhage/failure, DIC, Septic Shock, Acidosis, and End organ failure (Renal, Cardiac)]
