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Pediatrics Flashcards

1
Q

What is the most common upper airway disease in children?

A

Croup

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2
Q

What is the most common age group affected by croup?

A

Age 3 mos- 5yrs

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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

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4
Q

What is the most common organism that causes croup?

A

Parainfluenza virus (enveloped ss RNA)

Note: influenza, RSV can also cause croup

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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

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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

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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

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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)
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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.

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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

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11
Q

What is the most common organism associated with Bacterial tracheitis?

A

S. aureus

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12
Q

What age group is commonly affected by Bacterial tracheitis?

A

Children

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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
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14
Q

What is the treatment for bacterial tracheitis?

A

Dicloxacillin (PO)

Oxicillin, Nafcillin (IV)

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15
Q

How can epiglottitis and croup be distinguished. clinically?

A

Croup: Barky Cough
Epiglottitis: No cough

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16
Q

What age is most common for foreign body aspiration?

A

Children

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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

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18
Q

What anatomic sites are most common area of foreign body aspiration?

A

> 1 yr: Larynx

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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)

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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)

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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

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22
Q

What is the standard treatment for bronchiolitis?

A

Supportive care

Hospitalize If severe (tachypnes >60b/min) and try nebulized B2 agonist

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23
Q

What are some ways to prevent RSV?

A

Breastfeeding (IgA)

RSV IVIG or palivizumab (anti-RSV F protein Ab)

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24
Q

Who should receive RSV prophylaxis?

A

Preterm babies

Those with bronchopulmonary dysplasia

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25
Q

What is the age group that most commonly get viral pneumonia?

A

Age

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26
Q

What are common organisms associated with viral pneumonias?

A

RSV
Parainfluenza virus
Adenovirus

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27
Q

What age is common for presentation of bacterial pneumonia in children?

A

> 5y/o

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28
Q

What organisms are comonly associated with bacterial pneumonia in children?

A

S.pneumo
Mycoplasma
Chlamydia pneumoniae

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29
Q

What is distinct between viral and bacterial pneumonias on presentation?

A

Viral: URI BEFORE the pneumonia

Bacterial: acute onset high chills/fever, decreased breath sounds

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30
Q

What are some typical features associated with Chlamydia trachomatis pneumonia?

A 
Stacatto cough
Absence of fever/wheezing (unlike RSV)
Eosinophilia
Infants (1-3 mos)
\+/- Congenital at birth
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31
Q

What is the best way to distinguish Chlamydia pneumoniae from Mycoplasma pneumonia clinically?

A

It cannot be done.

Note: chlamydia pneumonia pneumonia is very different from that associated with chalmydia trachomatis

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32
Q

What is the best way to confirm Mycoplasma pneumoniae infection?

A

IgM titers against mycoplasma

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33
Q

If there are pneumatoceles (cavitations) present on CXR, what is the most likely organism?

A

S. aureus

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34
Q

What is the best initial test in dx’ing a child with possible pneumonia?

A

CXR

other tests include: CBC w/ diff, viral/mycoplasma IgM titers, Blood Cultures

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35
Q

What are the best treatment options for bacterial pneumonias?

A

Outpt: Amoxicillin (alt, cefuroxamine or amox/clavulanate)

Inpt: IV cefuroxamine

Chlamydia/Mycoplasma: Macrolide

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36
Q

What chromosome and common gene are affected in cystic fibrosis?

A 
Chromosome 7
CFTR gene (codes for Cl- channel)
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37
Q

What is the best initial test to dx CF?

A

Sweat Chloride test (x2 on separate days showing sweat Cl- >60mEq/L)

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38
Q

What are some common associated findings associated with Cystic Fibrosis?(9)

A 
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
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39
Q

What is the best initial test for a new born who failed to pass meconium in 48 hrs after birth?

A

Abdominal Xray (see microcolon)

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40
Q

What are two common organisms that infect the airway of pt with CF?

A

S. aureus
Pseudomonas
H. influenzae

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41
Q

At what age should PFT’s be initiated in pt with Cystic Fibrosis?

A

5 or 6 yrs of age

(PFT’s show an obstructive pattern initially (FEV1/FVC

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42
Q

What type of testing should all CF pts receive to guide therapy?

A

Genotyping -to determine presence of G551D mutation. If present can give Ivacaftor (VX-770) to restore CFTR function

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43
Q

What tests are used to monitor disease pregression?

A

CXR

PFTs

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44
Q

What treatments are shown to improve survival?

A

Ibuprofen

Azythromycin and other Ab’s during exacerbations

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45
Q

What Ab’s are used to treat CF -related infections?

A

Mild Disease: Macrolide, TMP/SMX, or Cipro

Documented S.aureus or Pseudomonas: Piperacillin+ Tobramycin or Ceftazidime

Resistant Pathogen: Inhaled Tobramycin

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46
Q

What immunizations should CF pts receive?

A

All immunizations plus pneumococcal and annual flu shot.

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47
Q

What is the most common cause of death in infants between 1-12 mos of age?

A

Sudden Infant Death Syndrome (SIDS)

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48
Q

What is the best test to detect apnea in an infant?

A

Polysomnography

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49
Q

What is the most common cause of inspiratory stridor in infants within first 2 ws -6mos of life?

A

Laryngomalacia

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50
Q

How is laryngomalacia diagnosed?

A

Clinical dx but can do Laryngoscopy to confirm (to see degree of stenosis)

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51
Q

What Laryngeal pathology is associated with meningomyelocele, hydrocephalus, and Arnold Chiari malformation?

A

Vocal Cord Paralysis

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52
Q

What is the best test to dx ?

A

Flexible Bronchoscopy

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53
Q

What are some common signs of CHF in infants?

A 
FEEDING problems:
difficulty feeding
sweating while feeding
easily fatigued (during feeding)
FTT/poor wt gain
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54
Q

What is the best initial test in working up a child with possible Congenital Heart Disease?

A

CXR (enlarged heart)
and
EKG (initially, LV hypertrophy)

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55
Q

What is the most accurate test for dx’ing CHD/CHF in a child

A

Echocardiography

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56
Q

What is the most common Congenital Heart Defect?

A

VSD

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57
Q

What type of murmur is associated with VSD?

A

Holosystolic +/- thrill (dependent upon size of the defect)

Note: smaller defects are more audible than larger ones.

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58
Q

What is a complication of VSD?

A

Eisenmenger Syndrome

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59
Q

What is the definitive treatment for Eisenmenger Syndrome?

A

Hear-Lung transplant

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60
Q

What is treatment for CHF in child?

A

Medications initially until Surgery is possible

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61
Q

What is a complication for any high flow lesion?

A

Endocarditis

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62
Q

What is the most common type of ASD?

A

Scundum type (area of foramen ovale)

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63
Q

What type of heart sounds/murmur is associated with ASD?

A

WIDE FIXED SPLIT S2 (increased flow through PV–>closes later)

Systolic Ejection Murmur @ L upper sternal border (flow through PV)

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64
Q

What is the treatment for a patent ASD?

A

Surgery if it does not close spontaneously by age 4 or 5yrs old

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65
Q

What chromosomal abnormality is commonly associated with Endocardial cushions?

A

Trisomy 21 (Down Syndrome)

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66
Q

What is the murmur associated with Endocardial Cushions?

A

Loud systolic murmur with thrill

Diastolic flow murmur

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67
Q

What is the mumur associated with Patent ductus arteriosus?

A

Continuous machine-like (to and fro)

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68
Q

What physical exam findings can be associated with PDA?

A

Wide Pulse pressure (Systolic-diastolic)

Bounding Pulses

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69
Q

What is the treatment for pt with PDA?

A

Preterm: Indomethacin (NSAID)

Term: Surgical Closure

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70
Q

What abnormality can be seen on CXR and EKG in a pt with Pulmonary Artery Stenosis?

A 
When severe CXR: 
Enlarged Rt Hrt
Decreased Pulm vascularity
Pulm Artery Dilation 
Darker (blacker) lung fields (b/c less flow)

EKG: Right Ventricular Hypertrophy

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71
Q

What murmur is associated with Pulmonic Stenosis?

A

Short, low-pitched with systolic ejection Click

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72
Q

What skeletal finding can be present in Aortic Stenosis adult type in a child?

A

Rib Knotching (d/t collateral circulation over time)

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73
Q

What is the treatment for Coarctation of the Aorta infantile type?

A

Infant/Neonate: Prostaglandin E1 infusion (keep DA patent) and surgery when stable

In older, treat HTN first then surgery

(Surgery is definitive)

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74
Q

What is the most common cyanotic congenital heart condition presenting beyond infancy?

A

Tetrology of Fallot

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75
Q

What are the associated anomalies of Tetrology of Fallot?

A

Pulmonary Stenosis (infundibulum just below valve)
Overriding Aorta
Right Vent Hypertrophy
VSD

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76
Q

What is a common finding associated with TOF?

A

Tet Spells (hypercyanotic attacks)

Increased resistance to blood flow to the lungs at the infundibulum

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77
Q

What might a child do to relieve a tet spell?

A

Flex knees/hip to chest of infant
Child can Squat

(increase SVR and reverse the shunt to increase pulmonary flow)

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78
Q

What heart sound/murmur are associated with TOF?

A

Single S2 and Harsh

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79
Q

What are some typical CXR findings associated with TOF?

A

CXR: Boot-shaped heart (d/t upslanting APEX) with blackened lung fields

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80
Q

How is TOF treated?

A

Oxygen
BBlockers
PGE1 (cyanotic at birth)
Surgery 4-12 mos

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81
Q

What is the most common cyanotic heart condition presenting in newborn period?

A

Transposition of Great Vessels (TPGV)

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82
Q

What metabolic disorder tends to be associated with infants dx’s with TPGV?

A

Maternal Diabetes

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83
Q

What heart sound can be associated with TPGV?

A

Loud, single S2

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84
Q

How is TPGV treated?

A

PGE1 to maintain patent Ductus Arteriosus

Surgery ASAP

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85
Q

When should prophylactic Ab’s be given to prevent Endocarditis?

A

Only prophylax for dental procedures iff:

Prosthetic Valves

Un-repaired/Persistent Congenital Heart Disease

H/o Endocarditis

Cardiac transplant with Valve problems

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86
Q

At what age should routine BP checks begin?

A

3yo (must check in all 4 extremities)

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87
Q

What cause of HTN should always be on differential for a pediatric pt?

A

Renal causes

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88
Q

Who should be worked up for secondary HTN?

A

Newborn: think renal thrombosis
Child: coarctation, renal parenchyma, endocrine, meds

Adolescent: Obesity, Renal–>Urinary

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89
Q

What tests should be ordered initially to evaluate a pediatric pt for HTN?

A 
CBC
Urinalysis/Cx
BUN/Cr
Electrolytes
Glc
Calcium
Uric Acid
Lipid Panel (Essential HTN and Family Hx)
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90
Q

What additional tests can be done to evaluate for specific etiologies of HTN?

A

Echocardiogram
Renal U/s
VoidingCystourethrogram
(if recurrent UTI

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91
Q

What is the standard of care to manage HTN in pediatric pt?

A

Obese: Lifestyle modifications

If Lifestyle modifications dont work–> Medications

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92
Q

What is the best initial medicinal therapy in pediatric pt with HTN?q

A

Diuretics or B-blockers

Can add Calcium Channel blockers if needed

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93
Q

What medication should be added if renal involvement?

A

ACE inhibitors

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94
Q

What is the most common cause of diarrhea in infancy?

A

Rotavirus (ds DNA)

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95
Q

What is the most common cause of bloody diarrhea?

A 
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
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96
Q

What is the best initial test for evaluating a pediatric pt with diarrhea?

A

Stool sample: cultures, wbc ct, blood, ouvum and parasites

C.diff toxin if h/o Ab use

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97
Q

What is the best initial treatment for a pediatric pt with diarrhea?

A

Hydration
Replace Fluids/electrolytes

(Never use antidarrheals in peds pt)

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98
Q

With which organisms should Ab’s be given for diarrhea in pediatric pt?

A

Shigella (TMP/SMX)
Campylobacter (Erythromycin if severe or dysentery )
Salmonells (

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99
Q

What is a complication that must be monitored for in pediatric pt with cryptosporidium-related diarrhea?

A

Malnutrition

100
Q

What is the most common cause of Acute Renal Failure in young children?

A

Hemolytic Uremic Syndrome (HUS)

101
Q

What organisms are associated with HUS?

A

E.coli O157:H7 (most common)

Shigella, Salmonella, Campy

102
Q

What tests should be ordered in work up for pt suspected of having HUS?

A 
CBC
Peripheral Smear
Urinalysis
Urine protein/cr ratio
Electrolytes
BUN/Cr
Coombs Test (neg)
103
Q

What long term management should take place for pt who had/recovered from HUS?

A

BP monitoring for 5yrs

BUN/Cr monitoring for 2-3 yrs

104
Q

What is next step ina pt suspected of having Giardiasis?

A

Duodenal Aspirate and Bx or Immunoassay

105
Q

What is the best initial test in the workup for malabsorption?

A

Sudan Black stain (stool)

Can also do serum trypsinogen screen to assess pancreatic problem

106
Q

What is the best test to confirm Malabsorption in a pediatric pt?

A

72-hr stool collection for fecal fat

107
Q

What is the best initial test for Protein malabsorption?

A

Spot stool Alpha-1-Antitrypsin level

Note, cannot evaluate protein malabsorption directly

108
Q

What additional tests should be done when managing malabsorption?

A

Vitamin/Mineral levels: Iron, Ca, Mg, Zn, VIt D, A, B12, folate

109
Q

What conditions are Celiac disease pts at increased risk of developing?

A

Osteoporosis (decreased Ca)

GI malignancies (T-cell lymphoma)

110
Q

What is the best initial test in dx’ing Celiac disease in a child?

A

Antitransglutaminase Ab

111
Q

What is the most accurate test for dx’ing Celiac disease?

A

Bx with Histology

112
Q

What is the next step in management for a child

A

H2 Blockers (first line medical therapy in children)

113
Q

What is the best initial treatment for children who present with symptoms of GERD?

A

Feeding technique and consistency adjustments

114
Q

What is the best initial test to dx GERD in a child?

A

Esophageal pH monitoring

115
Q

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?

A

Abdominal Ultrasound

Note: Hypochloremic Metabolic Alkalosis is pathagnomonic for Pyloric Stenosis

116
Q

What is the treatment fot Pyloric Stenosis?

A

Correct fluid/electrolytes first then

Pyloromyotomy

117
Q

What is a key distinguishing feature between Pyloric Stenosis and Duodenal Atresia?

A

Pyloric Stenosis: Non-bilious vomiting

Duodenal Atresia: Bilious vomiting

118
Q

What condition should be suspected in a child presenting with small bowel obstruction WITHOUT a h/o prior surgery?

A

Volvulus

119
Q

What should be suspected in an infant presenting with Bilious emesis with abdominal pain?

A

Malrotation or Volvulus

120
Q

What is the best initial test when malrotation or volvulus suspected?

A

Ultrasound (see SMA and SMV inversion and duodenal obstruction)

or

Barium Enema

121
Q

How is malrotation/volvulus treated?

A

Surgery

122
Q

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?

A

Tc-99 pentechnetate Scan (radionucleotide scan) to detect gastric mucosa in lower abdomen –>dx Meckel Diverticulum

123
Q

What are some common features associated with Meckel Diverticulum?

A 
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
Q

What is the embryonic origin of Meckel Diverticulum?

A

Omphalomesenteric Duct

125
Q

T/F: Meckel Diverticulum is a pseudodiverticulum?

A

FALSE: Meckel Diverticulum is true, all 3 bowel wall layers involved (mucosa, submucosa, muscularis propria)

126
Q

How is Meckel Diverticulum treated?

A

Surgery

127
Q

What is a complication associated with Meckel Diverticulum

A

Spontaneous Intussusception (diverticulum acts as lead point)

128
Q

What are typical features associated with the presentation of Intussusception?

A

Child

129
Q

What is the best initial test in a pt presenting with episodes of colicky abdominal pain current jelly stools?

A 
Abdominal Xray (r/o obstruction)
Air/Barium Enema (dx and rx intussusception)

[Manual/Surgery if enema does not reduce]

130
Q

What is the most common age to develop UTI in pediactric population?

A

Boys= 1yo

Girls>2yo (most girls will hv one UTI b/f age 5)

131
Q

What is the best initial test to dx UTI?

A

Urinalysis

132
Q

What is the most accurate test to dx UTI in pediatric population?

A

Urine Culture

133
Q

What is the best initial treatment for uncomplicated UTI in child?

A

Amoxicillin or TMP/SMX

134
Q

What is the best treatment for pyelonephritis in a child?

A

IV Ceftriaxone or

Ampicillin + gentamicin

135
Q

What age is contraindicated for use of sulfonamides and nitrofurantoin?

A
136
Q

What age is contraindicated for the use of quinolones?

A
137
Q

What is is contraindicated for the use of Tetracyclines?

A
138
Q

What is the next step in management for a child treated for UTI?

A

Repeat Urine culture 1 wk after Ab stopped (confirm sterile urine) Recheck periodically for 1-2 yrs

139
Q

What are the indications for a Voiding Cystourethrogram?and renal ultrasound in a child with UTI?

A 
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
Q

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?

A

Urinalysis/Cx

VCUG and Renal U/s

141
Q

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?

A

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
Q

When is surgery indicated for VUR?

A

UTI while on Ab prophylaxis
New scar develops
Ab’s dont help

143
Q

What is the most common presentation associated with Obstructive Uropathy in children?

A

Infection/Sepsis

144
Q

What is the most common cause of bladder obstruction in boys and what are 2 key findings?

A

Posterior Urethral Valves

walnut-shaped mass over pubic symphysis and weak urinary stream

145
Q

What is the most common cause of palpable abdominal mass in Newborns?

A

Hydronephrosis and Polycystic Kidney Disease

146
Q

What is the best initial test to dx Obstructive Uropathy?

A

VCUG and renal Ultrasound

147
Q

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?

A

Urinalysis
ASO titer
Complement (esp C3)

148
Q

What is the most accurate test for dx’ing PSGN (APGN)?

A

Anti-DNase Ag

149
Q

What is the best initial treatment for a pt with PSGN (APGN)?

A 
Antibiotics: Penicillin (Erythromycin only if allergic)
Supportive care (no antihypertensive meds in peds though!)
150
Q

What are some common clinical features associated with Berger’s Disease (IgA Nephropathy)?

A 
Gross Hematuria
Recent URI/GI illness
Mild proteinemia
HTN
NORMAL C3 complement
151
Q

What is the best test to dx Berger’s Disease?

A

Renal Bx

152
Q

What is the best treatment for Berger’s Disease?

A

BP control/Supportive care

153
Q

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?

A

Alport Syndrome

154
Q

What should be checked on physical exam in a pt suspected of having Alport Syndrome?

A

Eye exam for ocular abnormalities

155
Q

What renal abnormality is suggested in a pt with bilateral flank masses and hypoplasia?

A

Autosomal recessive Polycystic Kidney Disease (infantile type)

156
Q

What are some symptoms that should raise suspicion for PCKD (AR)?

A 
Potter's Sequence
Hypertension
Oliguria
Acute renal failure
with prenatal h/o Olighydramnios
157
Q

What is the best initial dx step for a pt suspected of having PCKD (AR)?

A

Ultrasound -Kidney and Liver

158
Q

What is the treatment for PCKD (AR)

A

Dialysis and transplant

159
Q

What is the most common form of persistent proteinuria in school-aged children and adolescents?

A

Orthostatic Proteinuria

160
Q

What is the most common age range for a child to present with Nephrotic Syndrome?

A

between 2-6y/o

161
Q

What is the next step in management for a 3yo child who presents with swollen eyes, urinalysis showing 3+proteinuria, serum albumin

A

Oral Steroids (Great response with Minimal Change Disease)

162
Q

How long should steroid therapy be continued when treating Minimal Change Disease?

A

Daily 4-6wks then taper by alternating days for 2-3 months without bx.

163
Q

What are the typical findings associated with Nephrotic Syndrome in pediatric pt?

A

Proteinuria (>40mg/m2/hr; Cr normal)

Hypoalbuminemia (

164
Q

What is the next step in management for a pt who has a relapse of symptoms while on steroids for minimal change disease?

A

Switch to
Cyclophosphamide, Cyclosporine, or
High-dose, pulsed methylprednisolone

165
Q

What are possible complications in a child with Nephrotic Syndrome?

A

Infection (must get Pneumococcal and Varicella)

Thromboembolism (loss of anticoag protein factors)

166
Q

What are the features associated with a presentation of CAH, 21-hydroxylase deficiency?

A 
Ambiguous genitalia (Masculinization of female genitalia)
Vomiting
Dehydration
Hyperkalemia
Hyponatremia
Hypoglycemia
167
Q

What are the metabolic derrangements associated with CAH-21hydroxylase deficiency?

A 
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
Q

What is the best initial test in dx’ing a pt with CAH?

A

Serum 17-Hydroxyprogesterone (elevated)

Can also test Cortisol (low), Renin (High),
Aldosterone (Low)

169
Q

What is the best confirmatory test to dx pt with CAH?

A

17 Hydroxyprogesterone before and after IV bolus of ACTH

170
Q

What is the best treatment for a pt with CAH?

A

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
Q

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?

A

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
Q

What is a common, life threatening complication of Kawasaki disease?

A

Coronary abnormalities

173
Q

What should the workup for Kawasaki diseaseinclude?

A

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
Q

What is the most likely dagnosis in a child b/w 2-8yo with Abdominal pain, palpable Purpura (maculopapular rash), Fever, and recent URI?

A

Henoch-Schonlein Purpura (HSP)

[small vessel vasculitis]

175
Q

What immune components are key factors in HSP?

A

IgA and C3 (deposit in arterioles, capillaries, venules)

176
Q

How are IgA Nephropathy (Berger’s) and HSP distinguished?

A

Berger’s mainly 20-30yo pt, renal only

HSP mainly children; systemic disease (renal, skin, connective tissues, joints, and GI)

177
Q

What medical conditions is HSP associated with?

A

Intussusception
Arthritis
Glomerularnephritis/nephrosis

178
Q

What is the best way to diagnose HSP?

A

H&P (purpura on legs/bottom, fever, abdominal pain, child with recent URI)

179
Q

What test can be done to support a dx of HSP?

A 
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
Q

How is HSP treated?

A

If GI or Renal sx: Steroids

If Anticardiolipin/Antiphospholipid Ab’s + and/or thrombosis occurs: Aspirin (antiplatelet)

181
Q

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?

A

NO Treatment. This is physiologic anemia and can last until about 3 months of age (due to EPO suppression at birth)

182
Q

At what age would you expect to see a baby present with iron deficiency anemia?

A

9-24 mos

183
Q

What is the treatment for Iron-def Anemia in pediatric pt?

A

Oral ferrous salts
Limit cow’s milk
Continue Iron replacement for 8 wks after Blood levels normalize to replenish stores

184
Q

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?

A

Lead Poisoning

185
Q

What is the best initial test to screen for Pb toxicity?

A

In high-risk pt: Blood Lead levels at 12 and 24 months (not finger prick)

186
Q

What is the maximum cut off for acceptable Blood Lead Level in a child?

A

5mcg/dL

187
Q

What labs/results can be associated with Pb toxicity?

A

CBC: Microcytic Hypochromic Anemia
Free Erythrocyte Porphyrins (FEP)
Peripheral Smear: Basophilic Stippling
XRAY (long Bones): Dense Lead Lines

188
Q

What is the next step in management whena child is found to have a blood lead level >15mcg/dL and >45mcg/dL?

A

> 15mcg/dL: Report to Health Department

>45mcg/dL: Chelation with (Calcium Disodium EDTA)

189
Q

What are the most common causes of mortality in pediatric cases of Sickle Cell Disease?

A

Infection(sepsis) and Acute Chest Syndrome

Acute Spelnic Sequestration

190
Q

What organisma are pts with sickle cell disease particularly susceptible to?

A

Encapsulated organisms (S.pneumo, H. influenza, N. meningitidis) d/t autoinfarction (usually by age 5)

191
Q

What is the best diagnostic test to dx and screen (newborn) for sickle cell disease?

A

Hb Electrophoresis

192
Q

When is the earliest a pt can be screened for sickle cell disease?

A

Prenatally (if both parents have trait)
CVS: 10-12 wks
Amniocentesis: 14-18 wks

193
Q

What is the indication for blood transfusion in pt with Sickle Cell disease?

A

Symptomatic Anemia (SOB, Chest pain)

194
Q

What is the indication for Hydroxyurea in pt with Sickle Cell Disease?

A

Symptomatic Anemia
3+ crises/year
Life-threatening compliations (stroke, acute chest syn, splenic crisis)

195
Q

What is the indication for Exchange transfusion in a pt. with Sickle Cell Disease?

A

Life-threatening complications

Before high-risk surgery

196
Q

What is the routine management for pts living with Sickle Cell Disease?

A

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
Q

What is the best initial test in the work up of a pt suspected of Thalassemia?

A

Hb Electrophoresis (also most specific)

198
Q

What additional tests should be included in dx’ing B-thalassemia?

A

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
Q

What Electrophoresis results ae expected in a pt with B-Thalassemia Major?

A

Increased HbF (Alpha tetramers d/t excess alpha globin chains)
Decreased/Absent HbA
Variable HbA2

200
Q

What findings can be associated with B-thalassemia Major?

A

Anemia, hypersplenism, cardiac decompensation. expanded medullary space (esp face and skull), extramedullary hematopoeisis, hepatosplenomegaly

201
Q

How should bacterial infections be managed in pt with sickle cell disease?

A

Aggressive Ab therapy

202
Q

How is B-Thalassemia major treated?

A

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
Q

What is included in routine management for a pt living with B-Thalassemia Major?

A

Folic Acid Supplementation
Immunizations
Daily Penicillin for prophylaxis
Growth Hormone replacement (excess Iron –>GH deficiency)

204
Q

What types of bleeding are associated with Platelet or Von Willebrand Factor(vWF) Deficiency/Dysfunction?

A

Mucosal bleeding [oropharynx, conjunctiva, epistaxis, vaginal-(menorrhagia)]

Skin (surface): Petechiae, small ecchymoses, purpura, ,

Post-op bleeding

205
Q

What type of bleeding is associated with Clotting Factor Deficiencies?

A

Deep Bleeds: Hemarthrosis, bleed into muscle, Large Ecchymoses, Hematoma

206
Q

For what components of platelet activation/aggregation is the glycoprotein IIb/IIIa receptor affinity increased upon platelet activation?

A

vWF and fibrinogen

207
Q

What are the best initial test to work up a pediatric pt with a bleeding disorder?

A

CBC (platelets, low platelets most common cause in kids)
Bleeding Time
PT(Extrinsic)
PTT(Intrinsic)

208
Q

What are the best confirmatory tests to work up bleeding disorders?

A 
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
Q

How should a mixing study be interpreted that shows correction of prolongation when normal plasma added to pt?

A

Clotting Factor deficiency

210
Q

How should a mixing study be interpreted that shows NO correction/partial correction of prolongation when normal plasma added to pt?

A

Inhibitor to clotting factor is present in pt sample

211
Q

How should a mixing study be interpreted that shows increased prolongation with clinical bleeding when normal plasma added to pt?

A

Antibody against a clotting factor is present in pt. sample

212
Q

How should a mixing study be interpreted that shows prolongation with a prolonged PTT and no bleeding when normal plasma added to pt?

A

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
Q

What is the next step in management for a pt with a h/o hemophilia who has been well controlled suddenly develops bleeding diathesis?

A

Order Mixing Study (results should indicate Factor inhibitor present)

214
Q

What is the treatment for a pt with Hemophilia A with minor bleeding?

A

DDAVP (desmopressin) +
Aminocaproic Acid or Tranexamic Acid
(the latter two are antifibrinolytics)

215
Q

What is the treatment for a pt with Hemophilia A with major bleeding?

A

Factor VIII replacement

216
Q

What is the treatment for a pt with Hemophilia B with minor bleeding?

A

Factor IX concentrates

217
Q

What is the treatment for Hemophilia B with major bleeding?

A

Factor IX concentrates

Note: all bleeds in Hemophilia B receive Factor IX concentrates

218
Q

What is the treatment for a pt with vWD subtype 1 with minor bleed?

A

DDAVP (desmopressin)

219
Q

What is the treatment for a pt with vWD subtypes 2 and 3 with major bleeds?

A

plasma-derived vWF with Factor VIII concentrates

220
Q

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?

A

Idopathic Thrombocytopenic Purpura (ITP)

221
Q

What is the underlying mechanism causing ITP?

A

AutoAb against platelet surface proteins

222
Q

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?

A

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
Q

What is the treatment for ITP?

A

Steroids (in mild disease) immediately
IVIG (life-threatening bleed)
Chronic ITP: Rituximab or Splenectomy

224
Q

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?

A

Reassurance
Manage the fever
Evaluate for meningitis (physical exam)

225
Q

What findings indicate an increased risk of epilepsy in a child with febrile seizure?

A 
Atypical seizure (>15 min, >1/day, focal findings)
FHx of epilepsy and first seizure
226
Q

What is a seizure disorder of infancy marked by clusters of mixed flexor/extensor spasms that persist for minutes with brief intervals between?

A

Down Syndrome (most common CNS disorder associated with West Syndrome (infantile Spasm)

227
Q

What is the EEG finding associated with West Syndrome?

A

Hypsarrhythmia (very high voltage slow waves with spike and sharp wave irregularly interspersed)

228
Q

What is the treatment for West Syndrome?

A

ACTH, prednisone, Vigabatrin, B6 (pyridoxine)

229
Q

What is the firstline treatment for a child with Absence seizures?

A

Ethosuxamide

230
Q

What is the first line treatment for Partial Seizures?

A

Carbamazepine or Valproic Acid

231
Q

What is the definition of fever without a focus in children?

A

Child38/100.4

Fever lasts 3wks)

232
Q

What are the indications for Ab in a child with Fever without a focus?

A

Documented rectal temp>100.4/38

WBC>15,000 with > 1500 Neutrophils +bands

233
Q

What is the workup for Fever without a focus in a Neonate?

A

Hospitalize
Panculture (blood, urine)
Prohylactic Ab: cover GBS, Listeria, E.coli Ampicillin/Sulbactam or 2/3 gen Cefalosporin)

234
Q

What is the most common organism associated with Fever without a focus in an infant?

A

Strep pneumo

235
Q

What is the treatment for Fever without a focus in an infant?

A

Well-appearing: IM Ceftriaxone (single dose)

Toxic Appearing: Empiric IV Ab

236
Q

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.

A

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
Q

What is the treatment for a child with Strep pneumo meningitis?

A

Penicillin or 3rd gen Cephalosporin for 10-14 dys

238
Q

What is the treatment for a child with Gram -neg (E.coli) meningitis?

A

Third gen Cephalosporin for 3wks

239
Q

What is the treatment for a child with empirically treated and no organism isolated meningitis?

A

Third gen Cephalosporin for 7-10 dys

240
Q

What is the treatment for a child with HiB meningitis?

A

Ampicillin for 7-10 dys plus Dexamethasone

241
Q

What is the treatment for a child with N. meningitidis meningitis?

A

Penicillin for 5-7 days

242
Q

What is the prophylactic treatment for bacterial meningitis and who should receive it?

A

Rifampin for N.meningitidis and HiB to all close contacts

243
Q

What is the most common complication of meningitis, esp with S. pneumo?

A

Hearing loss

244
Q

What are some complications associated with meningitis especially if treatment is delayed?

A

Clots, Mental retardation, neurologic dysfunction

245
Q

Seizure and persistent fever are complications of meningitis associated esp with which causal organism?

A

HiB

246
Q

What complications are associated with meningococcal meningitis?

A

Waterhouse Freidrichsen Syndrome [Adrenal helorrhage/failure, DIC, Septic Shock, Acidosis, and End organ failure (Renal, Cardiac)]

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