Falcon Review Pediatrics 1 Flashcards Preview

► Med Misc 06 > Falcon Review Pediatrics 1 > Flashcards

Flashcards in Falcon Review Pediatrics 1 Deck (284):
1

Why is breast feeding best

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

2

What supplementation is needed for breast feeding babies

Vitamin D by 2 months
Fluoride after 6 months (h20 dependent)

3

What disease contraindicate breast feeding

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

4

What are common medications that are contraindicated for breast feeding

1. Antineoplastics
2. Lithium
3. Chloramphenicol
4. Cyclosporine

5

Is mastitis a contraindication for breast feeding

NO

Actually part of the treatment

6

What are the language developmental milestones

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

7

What are the key gross motor developmental mile stones

6 months: sits
18 months: crawls up stairs

8

what are the key fine motor developmental mile stones

9 months: pincer grasp
15 months: 2 block tower

9

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

circle: 3
square: 4
triangle: 5

10

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

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

11

Is gynecomastia common in prepubertial boys

yes. Up to 50% of prepuberty boys

12

How long after initiation of menses will girls experience anovulatory periods

12-24 months

13

What is the first sign of puberty in males and females

Males: testicular enlargement
Females: thelarche

14

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

Boys: 9-14
Girls: 8-13

15

What is the most common cause of death in infants

SIDS
Sudden infant Death Syndrome

16

What are risk factors associated with SIDS

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

17

What is the best technique to reduce the risk of SIDS

back to sleep

18

At what temperature is fever defined

38C
100.4 F

19

What are the 2 most important details for sick kids

Age of the child
Height of the fever

20

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

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

21

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

Group B strep
E. Coli
Listeria

22

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

Strep pneumoniae
N. Meningitides
H. Influenza
Salmonella

23

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

Admitted, worked up and treated empirically with
1. Ampicillin for Listeria
2. Cefotaximine for GBS & E. Coli

24

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

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

25

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

No.

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

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

26

What is blepharitis

Inflammation of the lid margins.
Often Chronic
Not painful

27

What is a hordeolum

a Stye
Infection of the ciliary follicle
Painful and swollen

28

What is a chalazion

blocked meibomian gland
Classically not painful

May require surgical resection

29

What are the most common causes of conjunctivitis

Dacrostenosis
Chemical
Infection
Allergic

30

What is dacrostenosis

Congenital lacrimal duct obstruction

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

31

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

1. Decreased EOM
2. Proptosysis
3. Decreased visual acuity.

32

What is the most common cause of a Viral URI

Rhinovirus (#1)

parainfluenza, RSV, coronavirus

33

What are the signs and symptoms of a viral URI

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

34

What sinuses are infants born with

Ethmoid and maxillary

35

What are the causes of sinusitis

Strep Pneumoniae (#1)

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

36

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

Allergic rhinitis

37

What is the first line treatment for otitis media

Amoxicillin (# 1)

38

What is considered a treatment failure for otitis media

otalgia or gever persisting after 72 hours of treatment

39

What is a cholesteatoma

Epithelial outgrowth in tympanic membrane; may destroy temporal bone structures

40

Do bacteria prefer an acidic or basic environment to grow

Basic

41

How sensitive is a strep rapid antigen test

90%

42

What causes strep pharyngitis

GABHS

Streptococcus pyogenes

43

What is the main reason to treat against GABHS

Prevent rheumatic fever.

There is no prevention against glomerular nephritis

44

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

Scarlet fever

45

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

GBS
L. Monocytogenes
E. Coli

46

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

S. Pneumoniae
N. Meningitides
H. Influenza

47

What are the most common causes of meningitis in asplenic patients

Strep Pneumoniae
Salmonella

48

What are the three major types of meningitis

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

49

How do you differentiate the major types of meningitis

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

50

What are the most common causes of encephalitis

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)

51

What is the most common organism for osteomyelitis

Staph Aureus

52

What is the most common cause of osteomyelitis in neonates

GBS, gram negative enteric bacilli

53

What is the most common cause of osteomyelitis in sickle cell

Staph Aureus
Salmonella

54

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

Pasteurella multocida

55

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

Psuedomonas aeruginosa

56

What modalities are useful in detecting osteomyelitis

MRI 2-3 days
Xray 10-14 days

57

How do you guide antibiotic therapy for osteomyelitis

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

58

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

Septic arthritis

59

What bacteria is associated with cat scratch fever

Bartonella hensalae

60

What are the signs and symptoms of cat scratch fever

Tender nodes enlarged up to 2 months.

61

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

Warthin-Starry Stain

62

What is the treatment for bartonella hensalae

None. Usually resolves spontaneously

63

What is parinaud occuloglandular syndrome

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

64

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

Peritonsilar abscess

65

What kind of virus is influenza

RNA

66

What is used for treatment of influenza

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

67

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

staph aureus

68

What are the signs and symptoms of adenovirus

Fever
pharyngitis
conjuctivitis
rhinitis
diarrhea
hemorrhagic cystitis

69

What kind of virus is adenovirus

DNA

70

What are the differential for lesions on palms and soles

Coxsackie
Syphyllis
Rocky Mountain Spotted fever

Scabies in infants

71

What causes hand foot and mouth disease

Coxsackie A16

72

When is hand foot and mouth prevalent

summer-fall

73

What causes 5th's disease

Parvovirus B19

74

What is another name for 5ths disease

Erythema Infectiosum

75

What are the signs and symptoms of 5ths disease

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

76

What are the complications of parvovirus B19

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

Pregnancy infxn can induce hydrops fetalis

77

What is rubeola

Measles

78

What causes rubeola

Measles virus (RNA paramyxovirus)

79

What is the prodrome of rubeola

3 C's
Cough
Coryza
conjunctivitis

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

80

What is Roseola

Exanthem subitum

81

What causes roseola

HHV 6

82

What are the signs and symptoms of roseola

children

83

What is rubella

german measles

84

What is the incubation of rubella

14-21 days

85

What are the signs and symptoms of rubella

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

86

What are forrcheimer spots

rose spots on the soft palate

87

What happens with a rubella infxn during pregnancy

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

88

What causes Mononucleosis

EBV

89

What are signs and symptoms of mono

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

90

What causes whooping cough

Bordetella pertussis

91

What are the stages of whooping cough

Catarrhal Stage:
Paroxysmal stage
Convalescent stage

92

Define the catarrhal stage of whooping cough

1-2 weeks in duration
rhinorrhea
conjunctival injection
cough

93

Define the paroxysmal stage of whooping cough

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

94

Define the convalescent stage of whooping cough

Decreasing frequency of symptoms

95

What pathogen is associated with croup

Parainfluenza virus

96

What is a stridor

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.

97

How is the severity of croup scored

Westley criteria

98

What is the treatment for whooping cough

Erythromycin.

Does not shorten disease, but limits disease evolution and communicability

99

What is the significance of HBsAg

measure of active disease

Can indicate a persistent carrier state

100

What is the significance of HBcAg

Shows exposure to disease

used to differentiate previous infection that has resolved from a vaccine

101

What is the significance of HBeAg

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

Indication of highly infective state

102

What causes lyme disease

Borrelia burgdorferi

103

How is lyme disease transmitted

deer tick, endemic in New England

104

What are the stages of lyme disease

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

105

What is the treatment for lyme disease

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

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

106

What causes Rocky mountain spotted fever

Rickettsia Rickettsii

107

How is rocky mountain spotted fever transmitted

tick vector in the east coast and western states

108

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

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

109

what is the treatment for RMSF

Doxycycline for any age; delay can be fatal

110

What is the treatment for scabies

permethrin cream

111

What is the treatment for lice

permetherin cream

112

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

milk curd will easily wipe off

113

What causes hook worms

Ancylostoma duodenale

114

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

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

115

How are hook worms diagnosed

direct fecal examination for eggs

116

What is a complication of hook worms

Iron deficient anemia that can lead to protein malnutrition

117

What is the treatment for hook worms

mebendazole
Albendazole

118

What causes pinworms

Enterobius vermicularis

119

What are the signs and symptoms associated with pin worms

nocturnal anal or vaginal pruritis

120

What is the treatment for pin worms

mebendazole
albendazole

121

What is the problem with ascariasis lumbricoides

Colicky andominal pain
emesis
cough
hemoptysis
may cause intestinal obstruction

122

What is the treatment for ascariasis

mebedazole
albendazole

123

What is the life cycle of ascariasis

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

What causes histoplasmosis

histoplasma capsulatum found in soil contaminated by bird droppings

Common in Mississippi, Missouri, ohio river valleys

125

What is the treatment for histoplasmosis

Amphotericin B for a symptomatic presentation.

126

What is the most common childhood cancer

Acute Lymphoblastic leukemia

127

What are the signs and symptoms of ALL

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

128

What is used to diagnose ALL

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

Manual Diff
- Lymphoblast on smear

129

What is the treatment for ALL

Remission induction - chemotherapy

130

What is a complication of chemotherapy

Tumor lysis syndrome

131

What is tumor lysis syndrome and how do you treat it

Syndrome of:
hyperuricemia
hyperkalemia
hyperphosphatemia

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

132

What is the typical age of onset for ALL

3-4 yo

133

What is the typical age of onset for Hodgkin disease

older children and adolescents

134

What are the risk factors associated with hodgkins disease

immunodeficiency
EBV

135

What are the signs and symptoms of hodgkins disease

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

136

What is the key diagnostic feature for hodgkins disease

Reed-Sternberg Cell

137

What are the types of hodgkins lymphoma

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

138

What is the primary age associated with non-hodgkin lymphoma

Most common in children 7-11 years

139

What are the signs and symptoms of non-hodgkin lymphoma

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

What appearance on biopsy will burkitts lymphoma show

starry sky appearance

141

What is the treatment of non-hodgkin lymphoma

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

142

What are the major classifcations for brain tumors in children

1. Infatentorial (60%)
- 2 to 12 years old

2. Supratentoria (15%)
- 12

3. Midline (15%)

143

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

intracranial mass

144

What is the most common brain tumor of childhood

Astrocytoma

Most common for both Supratentorial and infratentorial

145

Where does a medulloblastoma originate

90% originate from the cerebellar vermis

roof of the 4th ventricle

146

What age is generally effected by medulloblastoma

2-10

147

Where does a brainstem glioma generally effect

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

148

What are the two types of Brain Stem Gliomas

1. Anaplastic: poor prognosis
2. Lowgrade focal: good prognosis

149

Discuss the frequency of craniophrayngioma

Most common supra-tentorial tumor after astrocytoma

150

What are the signs and symptoms of a craniopharyngioma

Endrocrine syndromes
- growth failure/hypothyroidism
- diabetes insipidus
- Adrenal insufficiency

Peripheral Vision loss (optic chiasm)

Hydrocephalus

151

What is used to diagnose craniopharyngioma

MRI is definitive

152

What is parinauds syndrome

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

What is an optic glioma

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

154

What age is typically effected by a Wilms tumor

age > 3

155

What is the most common renal neoplasm in childhood

Wilms Tumor

156

What are the signs and symptoms of wilms tumor

Asymptomatic abdominal /flank mass over the age of 3.

may have abdominal pain, vomitting, hypertension

157

What is a neuroblastoma

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

158

What age is typically effected with neuroblastoma

Less than 3

159

What are the signs and symptoms of a neuroblastoma

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

Opsoclonus-myoclonus (dancing eyes-dancing feet)

Periorbital ecchymosis (raccoon eyes)

160

How is a neuroblastoma diagnosed

VMA and HVA levels in urine
CT
Tumor biopsy

161

What is leukocoria

absent red reflex

162

What is the most common cause of leukocoria

congenital cataracts

163

What is the most serious cause of leukocoria

retinoblastoma

164

What is a vaccine

attenuated live or killed microorganisms given to induce immunity

165

What is a toxoid

modified bacterial toxin, now nontoxic, stimulated formation of antitoxins

166

What is active immunity

production of antibody

167

What is passive immunity

Preformed antibodies are given

168

What is herd immunity

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

i.e. Measles and Rubella

169

What are the live vaccines

MMR
Varicella

OPV (oral polio virus) not used in US

170

What vaccines are grown in egg media

MMR
Influenza

171

Is an egg allergy a contraindication to vaccines

NOT to MMR

NOT to influenza unless history of anaphylaxis or severe allergy

172

What vaccines contain neomycin

OPV
Varicella
MMR

173

What vaccines contain streptomycin

IPV
OPV

174

Which vaccines are thimerosal free

All of them

175

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

Give as many as possible

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

176

How does vaccination in immuncompromised patients change

only be concerned with live vaccines

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

177

What complications are associated with DTaP

Inconsolable crying, high pitched crying
Fever > 105
Rarely seizures

178

When is DTaP contraindicated

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

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

Contraindicated with severe immunodeficiency or prior anaphylaxis

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

180

What is the contraindication to HiB vaccine

anaphylaxis

181

What pneumococcus vaccine is recommended to all infants

13 valent conjugate

182

What children should receive the 23-valent polysaccharide vaccine

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

183

What is the contraindication to the pneumococcus vaccine

anaphylaxis

184

When is influenza vaccine contraindicated

Age less than 6 months

185

Who should receive the meningococcal vaccine

Asplenic patients over the age of 2
complement deficient patients

186

What are the contraindications to meningococcal vaccine

anaphylaxis
Conjugate - previous guillan-barre

187

What are the contraindications to varicella vaccine

Pregnancy
Anaphylaxis

Use caution with patients on salicylates: may induce reyes syndrome

188

What is reyes syndrome

Syndrome induced by salicylates with a virus:

Liver failure
Encepholapathy

189

What is the incubation period for varicella

10-21 days

190

When is post exposure prophylaxis indicated for varicella

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

191

What is included in the allergic triad

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

192

What is atopy

inherited tendency to have a persistent IgE response to allergens

193

What causes allergic rhinitis

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

may be seasonal or perennial

194

What are the signs and symptoms of allergic rhinitis

Sneezing, watery/mucoid rhinorrhea, nasal obstruction, itching

boggy edematous pale nasal mucosa

Nasal "salute" or allergic "shiners"

195

What is the treatment for allergic rhinitis

The key is allergen avoidance

Antihistamines, decongestants, consider nasal corticosteroids

196

What are the signs and symptoms of urticaria

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

IgE mediated response

197

What are some common causes for urticaria

Food
Drugs
Viruses
bacteria
malignancy

198

What is chronic urticaria

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

Typically can only treat symptomatically

199

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

Erythematous pruritic rash with crusting and scaling

200

What is the treatment for eczema

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

201

What are some complications associated with eczema

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

202

What are the 4 major types of immunodeficiency

B Cell
T Cell
Phagocyte
Complement

203

What are the clinical characteristics of humoral deficiency

Frequent recurrent pyogenic infections with extracellular encapsulated organism

Frequent bacterial otitis, sinusitis, pneumonia = sinopulmonary

204

What are the most common encapsulated pathogens

HiB
N. Meningitides
Strep pneumoniae

205

When will X-linked (Bruton) Agamaglobulinemia typically present

after 6-12 months when the maternal antibodies fall

206

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

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

Hypoplasia of tonsils and adenoids (no B cells)

207

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

prophylactic antibiotics and monthly IVIg

208

What is the most common humoral AB deficiency

IgA defficiency

209

What are the risk factors for IgA deficiency

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

210

What are the signs and symptoms of IgA deficiency

Recurrent respiratory tract and urinary tract infections, chronic diarrhea

211

What are complications that are associated with IgA deficiency

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

212

Discuss IgG subclass deficiency

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

Most pts with IgG2 def will have IgA deficiency

213

What are the signs and symptoms of an IgG subclass def

sinopulmonary infections, normal growth

typically only mild infections

214

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

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

What is anergy

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

216

What is DiGeorge syndrome

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

217

What are the signs and symptoms of DiGeorges syndrome

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

How is DiGeorge syndrome diagnosed

FISH

Microdeletion of chromosome 22q11.2

219

What are the types of SCID

1. X-linked: IL-2 receptor y-chain defect
2. Autosomal recessive: Adenosine deaminase deficiency

220

What are the signs and symptoms of SCID

serious/recurrent infections by 3-6 months of age

Often first indication of SCID is Pneumocystis carinii infection

221

What is the treatment of SCID

bone marrow transplant

222

What is Wiskott Aldrich Syndrome

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

What is the treatment for wiskott aldrich syndrome

bone marrow transplant

224

What are the signs and symptoms of Ataxia telangectasia

Autosomal recessive

Ataxia
telangectasia of eyes and skin
Chronic sinopulmonary disease
Endocrine abnormalities

children are often wheel chair bound by age 12

225

What is chronic granulomatous disease

X-linked disorder of neutrophils

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

226

What are the signs and symptoms of CGD

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

227

How is CGD diagnosed

Inability of affected cells to reduce nitroblue tetrazolium

228

When does the umbilical cord typically separate

7-10 days after birth

229

What is LAD

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

What are the signs and symptoms associated with LAD

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

231

What are the clinical characteristics of complement deficiencies

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

When does physiologic anemia occur in infants

3-6 months for full term infants
6-10 weeks for preterm infants

233

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

6-24 months

234

What are the risk factors for iron deficient anemia

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

235

What are the signs and symptoms of iron deficient anemia

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

236

What will the labs show for an iron deficient anemia

CBC: hypochromic, microcytic anemia

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

Increased: TIBC, free erythrocyte protoporphyrin level, RDW

237

What is the post conceptual age

Gestational age + chronological age

238

What will the labs show for hemolytic anemia

Decreased: hemoglobin, haptoglobin

Increased: reticulocyte count, indirect bilirubin from increased hemolysis

239

What are the risk factors for hemolytic anemia

Extrensic: DIC, Autoimmune hemolysis

Intrinsic: G6PD, Sickle cell, hereditary spherocytosis

240

What defect is associated with hereditary spherocytosis

RBC skeletal protein causing shape to be altered: SPECTRIN

241

What are the signs and symptoms associated with hereditary spherocytosis

Anemia, splenomegally, jaundice

Erythropoesis rate usually exceeds hemolysis rate

242

What will the MCV and MCHC be for hereditary spherocytosis

Normal or low MCV

Increased MCHC

243

What is the osmotic fragility test

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

What is the treatment for hereditary spherocytosis

Consider splenectomy

245

What is the consequence of a splenectomy

increased risk of sepsis, especially to encapsulated organism.

246

What is G6PD deficiency

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

X-linked

247

What is the treatment for G6PD

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

248

What is the defect with sickle cell disease

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

249

What are the signs and symptoms associated with sickle cell

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

When is sickle typically diagnosed

newborn. Part of the screening

251

What is the treatment for sickle cell

proper vaccines and immunizations

penicillin prophylaxis

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

Transfusions

Hydroxyurea

252

How does hydroxyurea benefit patients with sickle cell

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

253

What is the difference between Beta Thalassemia major and minor

Major is a complete absence of beta globin synthesis

Minor has one normal and one abnormal beta globin gene

254

What is the pathophysiology behind Beta Thalassemia

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

255

What are the signs and symptoms of Beta Thalassemia major

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

What are the signs and symptoms of Beta Thalassemia minor

mild hemolytic anemia with significant microcytosis usually less than 70fL

257

What is the normal range for MCV in kiddo's

70+Age (until age 10)

80-100 is normal adults

258

What is alpha thalassemia

Reduced synthesis of alpha globulin

259

What is alpha thalassemia one gene deletion

silent carrier

260

What is alpha thalassemia two gene deletion

alpha thal trait

mild hypochromic anemia with mild microcytosis

261

What is alpha thalassemia three gene deletion

Hb Barts (y) in infants

HbH diseas (B4) in adults

262

What is alpha thalassemia four gene deletion

Bart's disease; sever anemia;

incompatible with life

263

What is diamond blackfan syndrome

Red cell aplasia with boney defects

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

Elevated red cell adenosine deaminase

264

What is TEC

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

What is fanconi anemia

Autosomal recessive pancytopenia characterized by bone marrow failure

266

What are the signs and symptoms of fanconi anemia

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

267

What are the complications associated with fanconi anemia

AML

268

What is acquired aplastic anemia

pancytopenia

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

269

What is ITP

Idiopathic thrombocytopenia purpura

Most common cause of thrombocytopenia in childhood

Immune mediated

270

What are the signs and symptoms of ITP

epistaxis, bruising and petechial rash in otherwise well child;

271

What is kasabach-Merritt

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

What is hemophilia A

Factor VIII deficiency

X linked

most common

273

What bleeding times are associated with Hemophilia A

prolonged PTT

Normal platelet count, PT, bleeding

274

What is the treatment for Hemophilia A

DDAVP to increase factor VIII levels in mild cases

Factor VIII in severe cases

275

What are the signs and symptoms of Hemophilia A

Increased bleeding by 1 year:
injection
circumcision
hemarthrosis
excessive bruising

276

What is hemophilia B

Factor IX deficiency

X linked
Vitamin K dependent factor

277

What is the most common inherited bleeding disorder

Von Willebrands disease

Autosomal Dominant

278

What is the dysfunction in Von Willebrands disease

decreased production of vWF

279

what is the role of vWF

platelet adhesion
platelet aggregation
carrier for factor VIII

280

What are the signs and symptoms of von Willebrands disease

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

Hemarthrosis are RARE

281

What are the vitamin K dependent factors

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

282

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

PT

283

What can reduce vitamin K

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

284

What is DDAVP

desmopressin