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Med School Rotations > Falcon Review Pediatrics 1 > Flashcards

Falcon Review Pediatrics 1 Flashcards

1
Q

Why is breast feeding best

A

Immunologic factors such as IgA, Lactoglobulin, maternal macrophages

Decreased incidence of allergic disease, URI and otitis media

more rapid maternal return to pre pregnancy weight

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2
Q

What supplementation is needed for breast feeding babies

A

Vitamin D by 2 months

Fluoride after 6 months (h20 dependent)

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3
Q

What disease contraindicate breast feeding

A
  1. Active TB
  2. Syphilis
  3. HIV
  4. Varicella
  5. Galactosemia
  6. HSV breast lesion
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4
Q

What are common medications that are contraindicated for breast feeding

A
  1. Antineoplastics
  2. Lithium
  3. Chloramphenicol
  4. Cyclosporine
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5
Q

Is mastitis a contraindication for breast feeding

A

NO

Actually part of the treatment

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6
Q

What are the language developmental milestones

A 
2 months: social smile
4 months: laughs, coos
6 months: babbles
9 months: mama/dada, stranger anxiety
12 months: one word, specific mama/dada
15 months: one step command
18 months: 2 word phrases
24 months: 10 - 20 words, 2 step command
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7
Q

What are the key gross motor developmental mile stones

A

6 months: sits

18 months: crawls up stairs

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8
Q

what are the key fine motor developmental mile stones

A

9 months: pincer grasp

15 months: 2 block tower

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9
Q

What age should a child be able to draw a circle, square triangle

A

circle: 3
square: 4
triangle: 5

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10
Q

How much of a child should be understood at ages 2, 3, and 4

A

2 yo: 50% (2/4)
3 yo: 75% (3/4)
4 yo: 100% (4/4)

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11
Q

Is gynecomastia common in prepubertial boys

A

yes. Up to 50% of prepuberty boys

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12
Q

How long after initiation of menses will girls experience anovulatory periods

A

12-24 months

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13
Q

What is the first sign of puberty in males and females

A

Males: testicular enlargement
Females: thelarche

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14
Q

What is the average age of onset for puberty for boys and girls

A

Boys: 9-14
Girls: 8-13

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15
Q

What is the most common cause of death in infants

A

SIDS

Sudden infant Death Syndrome

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16
Q

What are risk factors associated with SIDS

A 
Prematurity
no prenatal care
maternal smoking
lower socioeconomic conditions
prone/side sleeping
Family history
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17
Q

What is the best technique to reduce the risk of SIDS

A

back to sleep

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18
Q

At what temperature is fever defined

A

38C

100.4 F

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19
Q

What are the 2 most important details for sick kids

A

Age of the child

Height of the fever

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20
Q

What is the approach for children less than 3 months of age with fever

A

Less than 1 month: hospital admission and R/O Sepsis workup

Less than 2 months: R/O sepsis w/u to include CBC, Blood Cx, Ua, Ur Cx, CSFa, CSFcx

Ages 2-3 months: depends on the clinical picture for CSFa and CSFcx

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21
Q

What are the most common causes of fever in infants less than 3 months

A

Group B strep
E. Coli
Listeria

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22
Q

What are the most common causes of fever in infants over 3 months of age

A

Strep pneumoniae
N. Meningitides
H. Influenza
Salmonella

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23
Q

How should a child less than 1 month with a fever be treated

A

Admitted, worked up and treated empirically with

  1. Ampicillin for Listeria
  2. Cefotaximine for GBS & E. Coli
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24
Q

What ages should a UTI be considered for children with a fever of unknown source

A

circumcised males: less than 6 months
uncircumcised males: less than 12 months
Females: less than 24 months

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25
Q

Can you give ceftriaxone to infants less than 1 month of age

A

No.

thought to displace bilirubin at binding site and induce neonatal jaundice.

Can use after one month old.
Less than one month use cefotaximine

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26
Q

What is blepharitis

A

Inflammation of the lid margins.
Often Chronic
Not painful

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27
Q

What is a hordeolum

A

a Stye
Infection of the ciliary follicle
Painful and swollen

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28
Q

What is a chalazion

A

blocked meibomian gland
Classically not painful

May require surgical resection

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29
Q

What are the most common causes of conjunctivitis

A

Dacrostenosis
Chemical
Infection
Allergic

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30
Q

What is dacrostenosis

A

Congenital lacrimal duct obstruction

May resolve in the first 24 hours. If not, can refer to optho for probe opening

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31
Q

When is a CT indicated in a patient with periorbital / orbital cellulitis

A
  1. Decreased EOM
  2. Proptosysis
  3. Decreased visual acuity.
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32
Q

What is the most common cause of a Viral URI

A

Rhinovirus (#1)

parainfluenza, RSV, coronavirus

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33
Q

What are the signs and symptoms of a viral URI

A

fever, nasal congestion / inflamed mucosa, rhinorrhea, sneezing, pharyngitis, malasise (5-7) days.

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34
Q

What sinuses are infants born with

A

Ethmoid and maxillary

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35
Q

What are the causes of sinusitis

A

Strep Pneumoniae (#1)

H. Influenza
S. Aureus (most common cause of chronic)
M. Catarallis
Anaerobes

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36
Q

If the turbinates are pale and boggy, what should be top of the differential

A

Allergic rhinitis

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37
Q

What is the first line treatment for otitis media

A

Amoxicillin (# 1)

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38
Q

What is considered a treatment failure for otitis media

A

otalgia or gever persisting after 72 hours of treatment

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39
Q

What is a cholesteatoma

A

Epithelial outgrowth in tympanic membrane; may destroy temporal bone structures

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40
Q

Do bacteria prefer an acidic or basic environment to grow

A

Basic

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41
Q

How sensitive is a strep rapid antigen test

A

90%

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42
Q

What causes strep pharyngitis

A

GABHS

Streptococcus pyogenes

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43
Q

What is the main reason to treat against GABHS

A

Prevent rheumatic fever.

There is no prevention against glomerular nephritis

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44
Q

What should be the first thought for a patient with a “sand paper rash”

A

Scarlet fever

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45
Q

What are the primary causes of meningitis for infants less than 3 months

A

GBS
L. Monocytogenes
E. Coli

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46
Q

What are the primary causes of meningitis for infants over 3 months

A

S. Pneumoniae
N. Meningitides
H. Influenza

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47
Q

What are the most common causes of meningitis in asplenic patients

A

Strep Pneumoniae

Salmonella

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48
Q

What are the three major types of meningitis

A

Pyogenic (bacterial)
Aseptic (Viral)
Granulomatous (TB)

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49
Q

How do you differentiate the major types of meningitis

A

Pyogenic: Increased cells with low glucose, positive gram stain

Aseptic: normal glucose, negative gram stain

Granulomatous: Low glucose, negative gram stain

HSV: significant amount of RBC’s

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50
Q

What are the most common causes of encephalitis

A
  1. Enterovirus (most common)
  2. Arboviral
    a. St. Louis
    b. Western Equine
    c. Eastern Equine
    d. Colorado tick fever
  3. HSV (associated with focal seizures)
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51
Q

What is the most common organism for osteomyelitis

A

Staph Aureus

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52
Q

What is the most common cause of osteomyelitis in neonates

A

GBS, gram negative enteric bacilli

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53
Q

What is the most common cause of osteomyelitis in sickle cell

A

Staph Aureus

Salmonella

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54
Q

What is the most common cause of osteomyelitis in dog/cat bites

A

Pasteurella multocida

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55
Q

What is the most common cause of osteomyelitis in puncture wound of the foot

A

Psuedomonas aeruginosa

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56
Q

What modalities are useful in detecting osteomyelitis

A

MRI 2-3 days

Xray 10-14 days

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57
Q

How do you guide antibiotic therapy for osteomyelitis

A

abx for 4-6 weeks while monitoring ESR. Continue tx until ESR has normalized

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58
Q

What should be suspected if the patient has painful joints and prefers the “frog leg” position

A

Septic arthritis

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59
Q

What bacteria is associated with cat scratch fever

A

Bartonella hensalae

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60
Q

What are the signs and symptoms of cat scratch fever

A

Tender nodes enlarged up to 2 months.

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61
Q

What stain is required to show show a tissue sample with bartonella hensalae

A

Warthin-Starry Stain

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62
Q

What is the treatment for bartonella hensalae

A

None. Usually resolves spontaneously

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63
Q

What is parinaud occuloglandular syndrome

A

Unilateral conjunctivitis, preauricular lymphadenopthy associated with running eyes after cat contact

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64
Q

If a patient is speaking and has a difficult time opening his mouth completely and sounds as if he is only whispering, what should you suspect

A

Peritonsilar abscess

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65
Q

What kind of virus is influenza

A

RNA

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66
Q

What is used for treatment of influenza

A

Amantadine/ rimantadine for sever cases (ineffective again B)
Oseltamivir: effective against both types

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67
Q

What is the most common cause of a secondary bacterial infection with the flu

A

staph aureus

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68
Q

What are the signs and symptoms of adenovirus

A 
Fever
pharyngitis
conjuctivitis
rhinitis
diarrhea
hemorrhagic cystitis
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69
Q

What kind of virus is adenovirus

A

DNA

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70
Q

What are the differential for lesions on palms and soles

A

Coxsackie
Syphyllis
Rocky Mountain Spotted fever

Scabies in infants

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71
Q

What causes hand foot and mouth disease

A

Coxsackie A16

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72
Q

When is hand foot and mouth prevalent

A

summer-fall

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73
Q

What causes 5th’s disease

A

Parvovirus B19

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74
Q

What is another name for 5ths disease

A

Erythema Infectiosum

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75
Q

What are the signs and symptoms of 5ths disease

A

low grade fever, phryngitis, slapped cheek rash followed by lacy appearing rash over the trunk and proximal extremities

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76
Q

What are the complications of parvovirus B19

A 
Reticulytopenia (temporary cessation of red cell generation)
Aplastic anemia (sickle cell)

Pregnancy infxn can induce hydrops fetalis

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77
Q

What is rubeola

A

Measles

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78
Q

What causes rubeola

A

Measles virus (RNA paramyxovirus)

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79
Q

What is the prodrome of rubeola

A

3 C’s
Cough
Coryza
conjunctivitis

Followed by high fever and maculopapular rash that starts at the head and spreads down

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80
Q

What is Roseola

A

Exanthem subitum

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81
Q

What causes roseola

A

HHV 6

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82
Q

What are the signs and symptoms of roseola

A

children < 5 yo
high fever followed by diffuse rose colored maculopapular rash (starts as papules on the trunk)
occipital lymphadenopathy
febrile seizures

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83
Q

What is rubella

A

german measles

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84
Q

What is the incubation of rubella

A

14-21 days

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85
Q

What are the signs and symptoms of rubella

A

Lymphadenopathy, maculopapular rash starting on the face and spreading down
Forccheimer spots

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86
Q

What are forrcheimer spots

A

rose spots on the soft palate

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87
Q

What happens with a rubella infxn during pregnancy

A

Congenital rubella syndrome (hearing loss, cataracts, HSM, blueberry muffin rash)

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88
Q

What causes Mononucleosis

A

EBV

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89
Q

What are signs and symptoms of mono

A 
pharyngitis
generalized lymphadenopathy
splenomegally
hepatomegaly
urticarial or maculopapular rash (associated with ampicillin/amoxicillin)
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90
Q

What causes whooping cough

A

Bordetella pertussis

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91
Q

What are the stages of whooping cough

A

Catarrhal Stage:
Paroxysmal stage
Convalescent stage

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92
Q

Define the catarrhal stage of whooping cough

A

1-2 weeks in duration
rhinorrhea
conjunctival injection
cough

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93
Q

Define the paroxysmal stage of whooping cough

A

2-4 weeks in duration
coughing spasms
inspiratory whoop
facial petechiae

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94
Q

Define the convalescent stage of whooping cough

A

Decreasing frequency of symptoms

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95
Q

What pathogen is associated with croup

A

Parainfluenza virus

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96
Q

What is a stridor

A

high-pitched wheezing sound resulting from turbulent air flow in the upper airway. Stridor is a physical sign which is produced by narrowed or obstructed airway path. It can be inspiratory, expiratory or biphasic. Inspiratory stridor is common.

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97
Q

How is the severity of croup scored

A

Westley criteria

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98
Q

What is the treatment for whooping cough

A

Erythromycin.

Does not shorten disease, but limits disease evolution and communicability

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99
Q

What is the significance of HBsAg

A

measure of active disease

Can indicate a persistent carrier state

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100
Q

What is the significance of HBcAg

A

Shows exposure to disease

used to differentiate previous infection that has resolved from a vaccine

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101
Q

What is the significance of HBeAg

A

Derived from HBcAg by proteolytic self cleavage; marker of active viral replication

Indication of highly infective state

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102
Q

What causes lyme disease

A

Borrelia burgdorferi

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103
Q

How is lyme disease transmitted

A

deer tick, endemic in New England

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104
Q

What are the stages of lyme disease

A

Stage 1: Localized erythema migraines 3-32 days after tick bite

Stage 2: Neurologic (Aseptic Meningitis, Bells Palsy) and Cardiac (myocarditis, heart block) 3-10 weeks after tick bite

Stage 3: Arthritis, peripheral neuropathy, encephalopathy 2-10 months after tick bite

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105
Q

What is the treatment for lyme disease

A

Stage I: Doxycycline (if patient is less than 8, use amoxicillin)

Stage II/III: IV Ceftriaxone or penicillin for 14-28 days

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106
Q

What causes Rocky mountain spotted fever

A

Rickettsia Rickettsii

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107
Q

How is rocky mountain spotted fever transmitted

A

tick vector in the east coast and western states

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108
Q

What are the signs and symptoms of RMSF (rocky mountain spotted fever)

A

Heachache
fever
blanching maculopapular rash which begins peripherally (including palms and soles) and spreads centrally; Rash eventually becomes petechial

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109
Q

what is the treatment for RMSF

A

Doxycycline for any age; delay can be fatal

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110
Q

What is the treatment for scabies

A

permethrin cream

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111
Q

What is the treatment for lice

A

permetherin cream

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112
Q

How can you tell the difference between milk curd and oral candida

A

milk curd will easily wipe off

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113
Q

What causes hook worms

A

Ancylostoma duodenale

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114
Q

What is the life cycle of hook worms (ancylostoma duodenal)

A

stool (eggs)
skin penetration
migrates to lungs via lymphatics
swallowed and attaches to intestinal mucosa

115
Q

How are hook worms diagnosed

A

direct fecal examination for eggs

116
Q

What is a complication of hook worms

A

Iron deficient anemia that can lead to protein malnutrition

117
Q

What is the treatment for hook worms

A

mebendazole

Albendazole

118
Q

What causes pinworms

A

Enterobius vermicularis

119
Q

What are the signs and symptoms associated with pin worms

A

nocturnal anal or vaginal pruritis

120
Q

What is the treatment for pin worms

A

mebendazole

albendazole

121
Q

What is the problem with ascariasis lumbricoides

A 
Colicky andominal pain
emesis
cough
hemoptysis
may cause intestinal obstruction
122
Q

What is the treatment for ascariasis

A

mebedazole

albendazole

123
Q

What is the life cycle of ascariasis

A

eggs ingested
larvae penetrate the intestinal wall
larvae migrate to lungs via the venous circulation
larvae enter alveolar spaces
larvae travel up broncial tree and are swallowed
adult worms fired (10-30 cm)

124
Q

What causes histoplasmosis

A

histoplasma capsulatum found in soil contaminated by bird droppings

Common in Mississippi, Missouri, ohio river valleys

125
Q

What is the treatment for histoplasmosis

A

Amphotericin B for a symptomatic presentation.

126
Q

What is the most common childhood cancer

A

Acute Lymphoblastic leukemia

127
Q

What are the signs and symptoms of ALL

A 
Fatigue
bone pain
fever
pallor
bleeding
headache
HSM
lympadenopathy
weight loss
gait disturbance
128
Q

What is used to diagnose ALL

A

CBC
- typically pancytopenia, though WBC can be elevated if leukemic cells are in circulation

Manual Diff
- Lymphoblast on smear

129
Q

What is the treatment for ALL

A

Remission induction - chemotherapy

130
Q

What is a complication of chemotherapy

A

Tumor lysis syndrome

131
Q

What is tumor lysis syndrome and how do you treat it

A

Syndrome of:
hyperuricemia
hyperkalemia
hyperphosphatemia

Treat with allopurinol, hydration, alkanalize the urine and monitory the heart

132
Q

What is the typical age of onset for ALL

A

3-4 yo

133
Q

What is the typical age of onset for Hodgkin disease

A

older children and adolescents

134
Q

What are the risk factors associated with hodgkins disease

A

immunodeficiency

EBV

135
Q

What are the signs and symptoms of hodgkins disease

A

localized adenopathy (cervical and supraclavicular 90%)
Fever
Sweats
weight loss (>10%)

136
Q

What is the key diagnostic feature for hodgkins disease

A

Reed-Sternberg Cell

137
Q

What are the types of hodgkins lymphoma

A

Nodular sclerosis
Mixed cellularity
Lymphocyte-rich
Lymphocyte depleted or not depleted

138
Q

What is the primary age associated with non-hodgkin lymphoma

A

Most common in children 7-11 years

139
Q

What are the signs and symptoms of non-hodgkin lymphoma

A

Abdominal mass
neck mass
Jaw mass or mass at other site

30% will present with RLQ pain. Always consider NHL for kiddo’s with RLQ pain

140
Q

What appearance on biopsy will burkitts lymphoma show

A

starry sky appearance

141
Q

What is the treatment of non-hodgkin lymphoma

A
  1. Surgical debulking
  2. Chemotherapy
  3. Consider radiotherapy for head and neck
142
Q

What are the major classifcations for brain tumors in children

A
  1. Infatentorial (60%)
    - 2 to 12 years old
  2. Supratentoria (15%)
    - < 2 and > 12
  3. Midline (15%)
143
Q

If a child experiences early morning wakenings with HA what should be suspected

A

intracranial mass

144
Q

What is the most common brain tumor of childhood

A

Astrocytoma

Most common for both Supratentorial and infratentorial

145
Q

Where does a medulloblastoma originate

A

90% originate from the cerebellar vermis

roof of the 4th ventricle

146
Q

What age is generally effected by medulloblastoma

A

2-10

147
Q

Where does a brainstem glioma generally effect

A

a glial cell tumor that infiltrates the pons and extend through the brainstem

148
Q

What are the two types of Brain Stem Gliomas

A
  1. Anaplastic: poor prognosis

2. Lowgrade focal: good prognosis

149
Q

Discuss the frequency of craniophrayngioma

A

Most common supra-tentorial tumor after astrocytoma

150
Q

What are the signs and symptoms of a craniopharyngioma

A

Endrocrine syndromes

  • growth failure/hypothyroidism
  • diabetes insipidus
  • Adrenal insufficiency

Peripheral Vision loss (optic chiasm)

Hydrocephalus

151
Q

What is used to diagnose craniopharyngioma

A

MRI is definitive

152
Q

What is parinauds syndrome

A

NOT TO BE CONFUSED WITH “PARINAUDS OCULOGLANDULAR SYNDROME”

AKA “Dorsal Midbrain Lesion”

Paralysis of upward gaze and a poor pupillary response to light. Caused by tumor pressure on the vertical gaze center to midbrain

153
Q

What is an optic glioma

A

low grade astrocytoma causing decreased visual acuity and pallor of the disks

154
Q

What age is typically effected by a Wilms tumor

A

age > 3

155
Q

What is the most common renal neoplasm in childhood

A

Wilms Tumor

156
Q

What are the signs and symptoms of wilms tumor

A

Asymptomatic abdominal /flank mass over the age of 3.

may have abdominal pain, vomitting, hypertension

157
Q

What is a neuroblastoma

A

Malignancy of the neural crest cells. 70% arise in abdomen with the adrenal as the primary site

158
Q

What age is typically effected with neuroblastoma

A

Less than 3

159
Q

What are the signs and symptoms of a neuroblastoma

A

Abdominal mass, kidneys typically NOT palpable (inferolaterally displaced by lesion), hypertension

Opsoclonus-myoclonus (dancing eyes-dancing feet)

Periorbital ecchymosis (raccoon eyes)

160
Q

How is a neuroblastoma diagnosed

A

VMA and HVA levels in urine
CT
Tumor biopsy

161
Q

What is leukocoria

A

absent red reflex

162
Q

What is the most common cause of leukocoria

A

congenital cataracts

163
Q

What is the most serious cause of leukocoria

A

retinoblastoma

164
Q

What is a vaccine

A

attenuated live or killed microorganisms given to induce immunity

165
Q

What is a toxoid

A

modified bacterial toxin, now nontoxic, stimulated formation of antitoxins

166
Q

What is active immunity

A

production of antibody

167
Q

What is passive immunity

A

Preformed antibodies are given

168
Q

What is herd immunity

A

When enough persons are immunized to prevent transmission of disease to unimmunized persons

i.e. Measles and Rubella

169
Q

What are the live vaccines

A

MMR
Varicella

OPV (oral polio virus) not used in US

170
Q

What vaccines are grown in egg media

A

MMR

Influenza

171
Q

Is an egg allergy a contraindication to vaccines

A

NOT to MMR

NOT to influenza unless history of anaphylaxis or severe allergy

172
Q

What vaccines contain neomycin

A

OPV
Varicella
MMR

173
Q

What vaccines contain streptomycin

A

IPV

OPV

174
Q

Which vaccines are thimerosal free

A

All of them

175
Q

What is the rule for catching up a child who has missed previously scheduled vaccines

A

Give as many as possible

Do not start over
Do not give two serial shots at one time

176
Q

How does vaccination in immuncompromised patients change

A

only be concerned with live vaccines

No OPV
Consider Varicella
Give MMR to mild and moderate immunocompromised, but hold in Severe

177
Q

What complications are associated with DTaP

A

Inconsolable crying, high pitched crying
Fever > 105
Rarely seizures

178
Q

When is DTaP contraindicated

A

Encephalopathy or seizures within 7 days of prior pertussis does not attributable to another cause
Anaphylaxis
unstable encepholapathy

Hold the pertussis; Still give the DT

179
Q

When is MMR contraindicated or when should it have a delayed administration

A

Contraindicated with severe immunodeficiency or prior anaphylaxis

Delayed for pregnancy and if pt has received IVIg in prior 3 months

180
Q

What is the contraindication to HiB vaccine

A

anaphylaxis

181
Q

What pneumococcus vaccine is recommended to all infants

A

13 valent conjugate

182
Q

What children should receive the 23-valent polysaccharide vaccine

A

children > 2 with sickle cell anemia, functional or anatomic asplenia

183
Q

What is the contraindication to the pneumococcus vaccine

A

anaphylaxis

184
Q

When is influenza vaccine contraindicated

A

Age less than 6 months

185
Q

Who should receive the meningococcal vaccine

A

Asplenic patients over the age of 2

complement deficient patients

186
Q

What are the contraindications to meningococcal vaccine

A

anaphylaxis

Conjugate - previous guillan-barre

187
Q

What are the contraindications to varicella vaccine

A

Pregnancy
Anaphylaxis

Use caution with patients on salicylates: may induce reyes syndrome

188
Q

What is reyes syndrome

A

Syndrome induced by salicylates with a virus:

Liver failure
Encepholapathy

189
Q

What is the incubation period for varicella

A

10-21 days

190
Q

When is post exposure prophylaxis indicated for varicella

A

immunocompromised patients

new borns born to mothers exposed 5 days prior to delivery and 2 days after delivery

191
Q

What is included in the allergic triad

A
  1. Allergic rhinitis
  2. Reactive airway disease / asthma
  3. Atopic dermatitis
192
Q

What is atopy

A

inherited tendency to have a persistent IgE response to allergens

193
Q

What causes allergic rhinitis

A

antigen stimulates IgE production which triggers histamine release from mast cells;

may be seasonal or perennial

194
Q

What are the signs and symptoms of allergic rhinitis

A

Sneezing, watery/mucoid rhinorrhea, nasal obstruction, itching

boggy edematous pale nasal mucosa

Nasal “salute” or allergic “shiners”

195
Q

What is the treatment for allergic rhinitis

A

The key is allergen avoidance

Antihistamines, decongestants, consider nasal corticosteroids

196
Q

What are the signs and symptoms of urticaria

A

Hives
Erythematous rasied pruritic lesions (Wheel and flare)
may be localized or generalized

IgE mediated response

197
Q

What are some common causes for urticaria

A 
Food
Drugs
Viruses
bacteria
malignancy
198
Q

What is chronic urticaria

A

Urticaria lasting longer than 6 weeks. Etiology often difficult to identify

Typically can only treat symptomatically

199
Q

What are the signs and symptoms of Atopic Dermatitis (eczema)

A

Erythematous pruritic rash with crusting and scaling

200
Q

What is the treatment for eczema

A

Primarily supportive with emollents and moisturizers
Decreased bathing
Antihistamines at night (even though it is not a histamine mediated reaction)
Topical corticosteroids

201
Q

What are some complications associated with eczema

A

Superinfections with Bacterial (staph or strep) as impetigo is most common or viral (herpetic)

202
Q

What are the 4 major types of immunodeficiency

A

B Cell
T Cell
Phagocyte
Complement

203
Q

What are the clinical characteristics of humoral deficiency

A

Frequent recurrent pyogenic infections with extracellular encapsulated organism

Frequent bacterial otitis, sinusitis, pneumonia = sinopulmonary

204
Q

What are the most common encapsulated pathogens

A

HiB
N. Meningitides
Strep pneumoniae

205
Q

When will X-linked (Bruton) Agamaglobulinemia typically present

A

after 6-12 months when the maternal antibodies fall

206
Q

What are the signs and symptoms of X-linked (Bruton) Agamaglobulinemia

A

pneumonia, otitis media, sinusitis caused by Strep Pnuemo or H. influenza

Hypoplasia of tonsils and adenoids (no B cells)

207
Q

What is the treatment for X-linked (Bruton) Agamaglobulinemia

A

prophylactic antibiotics and monthly IVIg

208
Q

What is the most common humoral AB deficiency

A

IgA defficiency

209
Q

What are the risk factors for IgA deficiency

A

Highly associated with SLE, RA, cancer, and celiac’s disease

210
Q

What are the signs and symptoms of IgA deficiency

A

Recurrent respiratory tract and urinary tract infections, chronic diarrhea

211
Q

What are complications that are associated with IgA deficiency

A

Anaphylaxis after blood transfusion due to development of anti-IgA AB

212
Q

Discuss IgG subclass deficiency

A

The type of deficiency will determine the type of frequent infections experienced

Most pts with IgG2 def will have IgA deficiency

213
Q

What are the signs and symptoms of an IgG subclass def

A

sinopulmonary infections, normal growth

typically only mild infections

214
Q

What are the clinical characteristics of disorders of T Cell mediated immunity

A

Frequent recurrent infections with opportunistic or low grade infection such as candida, mycobacteria, protozoa and viruses

Increased incidence of autoimmune disorders and malignancy

Anergy

215
Q

What is anergy

A

lack of an immune response to a specific antigen. Tested by a skin prick test

216
Q

What is DiGeorge syndrome

A

Hypoplasia/aplasia of thymus and parathyroid glands resulting from defect in development of the 3rd and 4th pharyngeal pouches

217
Q

What are the signs and symptoms of DiGeorges syndrome

A

Hypocalcemic seizures (typically in the neonate)
Recurrent or opportunistic infections
Congenital heart disease (pulmonary outflow tract and tetralogy of fallot)
Craniofacial abnormalities (hypertelorism, esophogeal atresia, bifid uvula and micrognathia)

218
Q

How is DiGeorge syndrome diagnosed

A

FISH

Microdeletion of chromosome 22q11.2

219
Q

What are the types of SCID

A
  1. X-linked: IL-2 receptor y-chain defect

2. Autosomal recessive: Adenosine deaminase deficiency

220
Q

What are the signs and symptoms of SCID

A

serious/recurrent infections by 3-6 months of age

Often first indication of SCID is Pneumocystis carinii infection

221
Q

What is the treatment of SCID

A

bone marrow transplant

222
Q

What is Wiskott Aldrich Syndrome

A

X-linked disorder of lymphocyte and megakaryocyte lineage

Triad of

  1. eczema
  2. thrombocytopenia (small platelets with short half life)
  3. Recurrent infections with encapsulated bacteria
223
Q

What is the treatment for wiskott aldrich syndrome

A

bone marrow transplant

224
Q

What are the signs and symptoms of Ataxia telangectasia

A

Autosomal recessive

Ataxia
telangectasia of eyes and skin
Chronic sinopulmonary disease
Endocrine abnormalities

children are often wheel chair bound by age 12

225
Q

What is chronic granulomatous disease

A

X-linked disorder of neutrophils

Unable to kill pathogens with respiratory burst. Dysfunction in NADPH oxidase enzyme complex

226
Q

What are the signs and symptoms of CGD

A 
failure to thrive, Chronic Diarrhea, persistent candidiasis
Skin and soft tissue infections
Lymphadenopathy and HSM
CBC with anemia of Chronic disease
Hypergammaglobinemia
227
Q

How is CGD diagnosed

A

Inability of affected cells to reduce nitroblue tetrazolium

228
Q

When does the umbilical cord typically separate

A

7-10 days after birth

229
Q

What is LAD

A

leukocyte adhesion defect

Defect in selectins and integrins: a disorder of glycosylation

Result is an increase in circulating leukocytes, but a decrease in function

Gammaglobulin is increased in response to infections, but overall effectiveness is impaired

230
Q

What are the signs and symptoms associated with LAD

A 
Delayed separation of umbilical cord
Necrotic periodontal infections
Leukocytosis
Neutrophilia, but no neutrophils in pus
Normal oxidative burst
231
Q

What are the clinical characteristics of complement deficiencies

A

recurrent bacterial infections with pyogenic extracellular, encapsulated organisms often involving the skin and respiratory tract

Increased susceptibility to recurrent meningococcal disease and disseminated N. gonorrhea

Increased incidence of autoimmune disease.

232
Q

When does physiologic anemia occur in infants

A

3-6 months for full term infants

6-10 weeks for preterm infants

233
Q

What age group of children are at risk for iron deficient anemia

A

6-24 months

234
Q

What are the risk factors for iron deficient anemia

A

Premature (inadequate iron stores)
Inadequate iron intake
Occult GI bleed (allergy, meckels)
Hookworms

235
Q

What are the signs and symptoms of iron deficient anemia

A

pallor
fatigue
tachycardia
systolic murmur; may progress to high output HF

236
Q

What will the labs show for an iron deficient anemia

A

CBC: hypochromic, microcytic anemia

Decreased: serum iron, reticulocyte count, ferritin, MCV and hemoglobin

Increased: TIBC, free erythrocyte protoporphyrin level, RDW

237
Q

What is the post conceptual age

A

Gestational age + chronological age

238
Q

What will the labs show for hemolytic anemia

A

Decreased: hemoglobin, haptoglobin

Increased: reticulocyte count, indirect bilirubin from increased hemolysis

239
Q

What are the risk factors for hemolytic anemia

A

Extrensic: DIC, Autoimmune hemolysis

Intrinsic: G6PD, Sickle cell, hereditary spherocytosis

240
Q

What defect is associated with hereditary spherocytosis

A

RBC skeletal protein causing shape to be altered: SPECTRIN

241
Q

What are the signs and symptoms associated with hereditary spherocytosis

A

Anemia, splenomegally, jaundice

Erythropoesis rate usually exceeds hemolysis rate

242
Q

What will the MCV and MCHC be for hereditary spherocytosis

A

Normal or low MCV

Increased MCHC

243
Q

What is the osmotic fragility test

A

ability of a RBC to take up water.

With a decreased surface area per unit volume, it will have a decreased ability to take up water

This test is abnormal with hereditary spherocytosis

244
Q

What is the treatment for hereditary spherocytosis

A

Consider splenectomy

245
Q

What is the consequence of a splenectomy

A

increased risk of sepsis, especially to encapsulated organism.

246
Q

What is G6PD deficiency

A

Normally glucose is metabolized through the hexose-monophosphate shunt to reduce glutathione and protect the red cell from oxidant stress.

X-linked

247
Q

What is the treatment for G6PD

A

avoidance of triggers: sulfa drugs, fava beans, antimalarials, high dose aspirin

248
Q

What is the defect with sickle cell disease

A

substitution of valine for glutamine at the 6th position of the B chain

249
Q

What are the signs and symptoms associated with sickle cell

A

generally assymptomatic until 6 months of age when maternal hemoglobin is lost.

6 months - 5 years: spleen autoinfarcts, and will have an increase infection from encapsulated organisms

Splenic sequestration: acute splenic engorgement, can lead to shock/death

vasoocclusive pain crisis

Gall stones from increased hemolysis of blood

250
Q

When is sickle typically diagnosed

A

newborn. Part of the screening

251
Q

What is the treatment for sickle cell

A

proper vaccines and immunizations

penicillin prophylaxis

Agressive manage of fever (Abx) and Pain (hydration)

Transfusions

Hydroxyurea

252
Q

How does hydroxyurea benefit patients with sickle cell

A

it increases production of HbF which deceases the percentage of blood that sickles

253
Q

What is the difference between Beta Thalassemia major and minor

A

Major is a complete absence of beta globin synthesis

Minor has one normal and one abnormal beta globin gene

254
Q

What is the pathophysiology behind Beta Thalassemia

A

Excess alpha chins don’t form tetramers, but adhere to RBC membranes damaging them.

255
Q

What are the signs and symptoms of Beta Thalassemia major

A

Sever progressive hemolytic anemia in the second 6 months of life after HbF fades (Cooley’s Anemia)

HSM, typically massive
Chipmunk facies from extramedullary erythropoesis
skin with copper color b/c of combination of pallor, icterus, melanin deposition

256
Q

What are the signs and symptoms of Beta Thalassemia minor

A

mild hemolytic anemia with significant microcytosis usually less than 70fL

257
Q

What is the normal range for MCV in kiddo’s

A

70+Age (until age 10)

80-100 is normal adults

258
Q

What is alpha thalassemia

A

Reduced synthesis of alpha globulin

259
Q

What is alpha thalassemia one gene deletion

A

silent carrier

260
Q

What is alpha thalassemia two gene deletion

A

alpha thal trait

mild hypochromic anemia with mild microcytosis

261
Q

What is alpha thalassemia three gene deletion

A

Hb Barts (y) in infants

HbH diseas (B4) in adults

262
Q

What is alpha thalassemia four gene deletion

A

Bart’s disease; sever anemia;

incompatible with life

263
Q

What is diamond blackfan syndrome

A

Red cell aplasia with boney defects

Bony abnormalities of thumbs (triphalangeal)
Webbed neck
Shield chest
Cleft lip

Elevated red cell adenosine deaminase

264
Q

What is TEC

A

transient Erythroblastopenia of Childhood

Acquired pure red cell aplasia between 6 months and 5 years caused by bone marrow suppression (likely viral induced)

Normal adenosine deaminase

265
Q

What is fanconi anemia

A

Autosomal recessive pancytopenia characterized by bone marrow failure

266
Q

What are the signs and symptoms of fanconi anemia

A

Pancytopenia
Congenital abnormalities - short stature, microcephaly, microphthalmia, hearing loss, absent radii and thumbs, hyperpigmentation

267
Q

What are the complications associated with fanconi anemia

A

AML

268
Q

What is acquired aplastic anemia

A

pancytopenia

typically associated with: 
Radiation
Drugs (chloramphenicol)
infections (parvovirus B19)
50% idiopathic
269
Q

What is ITP

A

Idiopathic thrombocytopenia purpura

Most common cause of thrombocytopenia in childhood

Immune mediated

270
Q

What are the signs and symptoms of ITP

A

epistaxis, bruising and petechial rash in otherwise well child; <1% have CNS bleed

No spleenomegaly

271
Q

What is kasabach-Merritt

A

Rapidly enlarging cavernous hemangioma (usually cutaneous) an a consumptive coagulatopathy resulting in thrombocytopenia - stagnant blood flow in low-flow hemangioma leads to consumption of coagulation proteins

272
Q

What is hemophilia A

A

Factor VIII deficiency

X linked

most common

273
Q

What bleeding times are associated with Hemophilia A

A

prolonged PTT

Normal platelet count, PT, bleeding

274
Q

What is the treatment for Hemophilia A

A

DDAVP to increase factor VIII levels in mild cases

Factor VIII in severe cases

275
Q

What are the signs and symptoms of Hemophilia A

A 
Increased bleeding by 1 year: 
injection
circumcision
hemarthrosis
excessive bruising
276
Q

What is hemophilia B

A

Factor IX deficiency

X linked
Vitamin K dependent factor

277
Q

What is the most common inherited bleeding disorder

A

Von Willebrands disease

Autosomal Dominant

278
Q

What is the dysfunction in Von Willebrands disease

A

decreased production of vWF

279
Q

what is the role of vWF

A

platelet adhesion
platelet aggregation
carrier for factor VIII

280
Q

What are the signs and symptoms of von Willebrands disease

A

prolonged bleeding from cuts, nose bleeds, menorrhagia, gum bleeding

Hemarthrosis are RARE

281
Q

What are the vitamin K dependent factors

A

2, 7, 9, 10 and Protein C and S

282
Q

With a vitamin K deficiency, what is a better indicator, PT or PTT?

A

PT

283
Q

What can reduce vitamin K

A

Mal absorptive
lack of neonatal injection
Warfarin
Antibiotic suppression of bacteria producing vit K

284
Q

What is DDAVP

A

desmopressin

Med School Rotations (26 decks)

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