Peds Flashcards
Metabolic Disorders ?
Proprionic acidemia
Methylmalonic acidemia
Isovaleric acidemia
3-Methylcrotonyl- CoA-
carboxylase deficiency
3-Hydroxy-3-methyglutaric aciduria
Holocarboxylase synthase deficiency
Beta-ketothiolase deficiency
Glutaric acidemia type I
Fatty Acid Oxidation Disorders ?
Carnitine uptake defect/carnitine transport defect
Medium- chain acyl-CoA dehydrogenase deficiency
Very long-chain acyl-CoA dehydrogenase deficiency
Long-chain L-3 hydroxylacyl-CoA dehydrogenase deficinecy
Trifunctional protein deficiency
Amino Acid Disorders ?
Argininosuccinic aciduria
Citrullinemia, type I
Maple syrup urine disease
Homocystinuria
Classic phenylketonuria
Tyrosinemia, type I
Endocrine Disorders ?
Congenital adrenal hyperplasia
Primary congenital hypothyroidism
Hemoglobinopathies ?
Sickle cell anemia
Beta-thalassemia
S,C disease
Other tests ?
Biotinidase deficiency
Critical congenital heart disease
Cystic fibrosis
Classic galactosemia
Glycogen storage disease, type II
Hearing loss
Severe combined immunodeficiencies
Recommended in infants ?
Born to mothers with gestational diabetes or DM
Large infants (>3969 g)
Small infants (<2608 g)
Premature (<37 weeks)
Smaller twin (sizes are discordant)
Polycythemia (>70% Hct)
Hypothermia
Low apgar scores (<5 at one min, <6 at five minutes)
Stress (sepsis, RDS)
Lead: CDC recommends universal lead screening despite risk factors for children ages _______ and targeted screening for older children at risk.
1 and 2
High levels of lead can cause ?
seizures or coma
Lead: Even blood levels less than __ have been correlated with lower intelligence quotients
10
Lead: < 10 ?
stunt bone growth as a consequence of decreased vit. D metabolsim
Lead: >10 ?
slow cognitive and behavioral development
Lead: >20 ?
delayed nerve conduction
Lead: > 40 ?
decrease hemoglobin synthesis
Lead: > 70 ?
anemia with acute poisoning
Lead: 100-150 ?
encephalopathy
Lead: >380 ?
convulsions, coma, death
Lead Poisoning: ____ mcg/dL is elevated and should be prompt for repeat screening
> 5
Lead Poisoning: >45 mcg/dL = ?
chelation therapy indicated
Lead Poisoning: > 70 mcg/dL = ?
immediate emergent treatment
Most common nutritional deficiency in the US ?
Iron Deficiency Anemia
Iron Deficiency Anemia severe causes ?
Anemia
Behavioral problems
Cognitive effects
Abnormal sleep cycles
Risk factors for IDA ?
Low birth weight
Pregnancy with multiples
IDA in the mother
Use of cow’s milk before 12 months of age
Low iron diet
Chronic illness
Restricted diets
IDA: Universal Screening at __ months by Hgb or Hct
12
If child is anemic, further tests may be needed like what ?
CBC
Iron studies
Hgb electrophoresis
Etc…..
TB: Screening with TST for high-risk patients include ?
Born outside of US? (TST for Africa, Asia, Latin America, or Eastern Europe)
Traveled outside the US greater than one week and staying with friends and family.
Exposed to active TB?
Close contact with a person with postive TST?
HepB: Vaccine - composition ?
Inactive subunit of Hep B virus
HepB: Vaccine - immunity life ?
Usually for life
Occasionally needs a booster
HepB: Vaccine - contraindications ?
none
HepB: Vaccine - Major adverse reactions ?
erythema multiforme
erythema nodosum
Stevens-Johnson syndrome
HepB: Vaccine - Common reactions ?
Irritability
fever
diarrhea
HepB: Vaccine - Dosing ?
Begins at birth
3-4 shots over 6 months (Recombivax, Engerix)
Central elements to reducing Hep B burden in the US ?
Immunizations of all infants beginning at birth
Routine screening of pregnant women
HBIG to all infants born of infected mothers
Vaccination
Rotavirus - vaccine: composition ?
live, oral, attenuated rotavirus
Rotavirus - vaccine: immunity life ?
No clear data on immunity life
For first year after vaccination
85%-98% protective against severe rotavirus disease
74%-87% protective against rotavirus disease of any severity
Rotavirus - vaccine: contrindications ?
Acute diarrhea
Severe immunodeficiency
Rotavirus - vaccine: major adverse reactions ?
intussusception
Kawasaki dz
ITP
Rotavirus - vaccine: common reactions ?
vomiting
irritability
diarrhea
flatulence
Rotavirus - vaccine: dosing ?
(Rotateq, Rotarix)
2 months AND
4 months
Rotavirus - vaccine: considerations ?
Viral antigens can be detected in stool
CBC usually shows normal WBC count
Patients may be hypernatremic
May develop a metabolic acidosis
Urine can show ketones
DTaP, Tdap - vaccine: composition ?
Diphtheria toxoid
Tetanus toxoid
Acellular pertussis
DTaP, Tdap - vaccine: immunity life ?
10 years
DTaP, Tdap - vaccine: contraindications ?
neurologic disorder, progressive
infantile spasms
uncontrolled epilepsy
DTaP, Tdap - vaccine: major adverse reactions ?
Seizures
hypotonic-hyporesponsive episode
Guillain-Barre syndrome
DTaP, Tdap - vaccine: common reactions ?
irritability
drowsiness
DTaP - vaccine: dosing ?
(higher dose diphtheria and pertussis) [Pediarix, TriHIBit)
5 shots
2 months 4 months 6 months 15-18 months 4-6 years
Tdap - vaccine: dosing ?
Tdap (Boostrix)
Booster every 10 years
Tetanus needs booster every 5 with dirty wounds
Diptheria acute infections with ?
Corynebacterium diptheriae: gram + club shaped rods
Diptheria facts ?
Hemolytic anemia can occur
Thrombocytopenia is frequently associated.
Diagnosis is clinical
Cultures are difficult to grow and require specialized media
Tetanus caused by ?
Clostridium tetani
Clostridium tetani
shape ?
Drumstick-shaped gram + bacilli
Tetanus dx. ?
clinical
Anaerobic culture and microscopic examination can be helpful but are often difficult
Pertussis considerations ?
Elevated WBC count ~20-30,000 with 70-80% lymphocytes
Identification by culture or PCR from nasal swab is the definitive diagnosis
Culture requires specific media
PCR is more commonly used
ELISA for detection of antibodies (may be unreliable in previously immunized patients)
Hib - vaccine: composition ?
Hib polysaccharide protein
Hib - vaccine: immunity life ?
usually for life
Hib - vaccine: contraindications ?
Children under six weeks
Hypersensitivity to tetanus toxoid
Hib - vaccine: major adverse reactions ?
angioedema
seizures
hypotonic-hyporesponsive episode
Hib - vaccine: common reactions ?
fever
fussiness
Hib - vaccine: dosing ?
(PedVaxHib)
3-4 doses
2 months
4 months
(6 months depending on formulation)
12-15 months
Haemophilus influenza B lab findings ?
WBC count = elevated with +/- left shift
Blood culture is frequently positive
Culture of aspirated pus or fluid from involved site (ie; septic arthritis)
CSF smear may show pleomorphic gram (-) rods
PCV13 (Pneumococcal) - vaccine: composition ?
conjugaated pneumococcal polysaccharides
13 strains of polysaccharides (was 7)
PCV13 (Pneumococcal) - vaccine: immunity life ?
Until 18 years old
Also given to adults:
Over 19 and immunocompromised OR
Over 65 and have not had PCV13
PCV13 (Pneumococcal) - vaccine: contraindications ?
none
PCV13 (Pneumococcal) - vaccine: major adverse reactions ?
anaphylaxis/anaphylactoid rxn
angioedema
erythema multiforme
PCV13 (Pneumococcal) - vaccine: common reactions ?
irritability
altered sleep patterns
fatigue
A 23 strain version (PPSV23) is not used in ________.
children
PCV13 (Pneumococcal) - vaccine: also given to adults if ?
Over 19 and immunocompromised OR
Over 65 and have not had PCV13
PCV13 (Pneumococcal) - vaccine: dosing ?
At least 4 doses
2 months
4 months
6 months
12-15 months
(>24 months depends on previous formulation administered and risk factors)
Inactivated poliovirus (IPV) - vaccine: composition ?
Polio antigens
Inactivated poliovirus (IPV) - vaccine: immunity life ?
Usually life
Inactivated poliovirus (IPV) - vaccine: contraindications ?
Febrile illness
Inactivated poliovirus (IPV) - vaccine: major adverse reactions ?
seizures
Inactivated poliovirus (IPV) - vaccine: common reactions ?
irritability
fatigue
Inactivated poliovirus (IPV) - vaccine: dosing ?
4 doses
2m
4m
6-18m
4-6y
Influenza - vaccine: composition ?
Usually trivalent grown in eggs, purified surface antigens
Usually contains A and B
Influenza - vaccine: immunity life ?
1 year
Influenza - vaccine: contraindications ?
Egg allergy
Guillan Barre (caution)
Influenza - vaccine: major adverse reactions ?
anaphylaxis
bronchospasm
Stevens-Johnson syndrome
neurologic disorders
Guillain-Barre syndrome
Influenza - vaccine: common reactions ?
myalgia
fatigue
irritability
Influenza - vaccine: dosing ?
Once yearly
Influenza CXR ?
Nonspecific findings
Peribronchial thickening
Diffuse interstitial infiltrates
hyperaeration
Influenza Lab tests ?
Rapid flu antigen from nasal swab
Influenza virus can be found by ?
DFA antibody staining of NP epithelial cells
ELISA
PCR
Culture
MMR - vaccine: composition ?
lyophilized virus grown in chicken embryos
MMR - vaccine: immunity life ?
life
MMR - vaccine: contraindications ?
hypersens. to gelatin
anaphylactic
anaphylactoid rxn to neomycin
immunocompromised pts
tuberculosis, active untreated
MMR - vaccine: major adverse reactions ?
erythema multiforme
Stevens-Johnson syndrome
atypical measles
MMR - vaccine: common reactions ?
arthralgia/arthritis
fever
urticaria
rash, measles-like
MMR - vaccine: dosing ?
2 doses
12-15m
4-6 yrs
Measles hx. ?
Lymphopenia/Leukopenia
high fever 104
runny nose, cough,
red watery eyes
Measles DS ?
Measles IgM drawn 3 days after the onset of rash
Fluorescent antibody staining of NP cells for direct detection of antigen
PCR of oropharyngeal secretions or urine can show positive results up to 5 days before symptoms begin.
Mumps DS ?
Viral culture or PCR of saliva, throat, urine, or CSF may be positive for one week after onset of symptoms
Single positive IgM is definitive diagnosis
Rubella DS ?
Leukopenia common
Thrombocytopenia common
4 fold rise in antibody titer in serum drawn 1-2 weeks apart
Positive IgM titers indicate acute infection
Rubella congenital infection is associated with ?
Low platelets
Abnl LFTs
CSF pleocytosis
Hemolytic anemia
Varicella - vaccine: composition ?
live attenuated varicella virus
Varicella - vaccine: immunity life ?
life
Varicella - vaccine: contraindication ?
hypersens. to gelatin
anaphylactic or anaphylactoid rxn to neomycin
immunocompromised pts tuberculosis,
active untreated pregnancy or planned pregnancy w/in 4wk
Varicella - vaccine: major adverse reactions ?
Stevens-Johnson syndrome
erythema multiforme
Henoch-Schonlein purpura
herpes zoster (shingles)
Varicella - vaccine: common reactions ?
fever rash,
varicella-like
URI sx
Varicella - vaccine: dosing ?
2 doses
12-15 months
4-6 years
Varicella lab tests ?
Leukocyte decrease
Fluorescent antibody tests of a lesion smear
PCR is definitive
HepA - vaccine: composition ?
Hep A antigens
HepA - vaccine: immunity life ?
life
HepA - vaccine: contraindications ?
Neomycin hypersensitivity
HepA - vaccine: major adverse reactions ?
angioedema
erythema multiforme
hepatotoxicity
thrombocytopenia
neurotoxicity
HepA - vaccine: common reactions ?
irritability
anorexia
fatigue
HepA - vaccine: dosing ?
2 doses
12-24 months
Hepatitis A lab findings ?
Bilirubin is elevated
LFTs are elevated
+/- prolongation of the PT
Definitive diagnosis made by positive anti-HAV IgM (anti-HAV IgG persists after infection resolves)
Meningococcal - vaccine: composition ?
4 conjugated polysaccharides
Meningococcal - vaccine: dosing ?
2 doses
11-12 years
16-18
Meningococcal lab findings ?
WBC count low or elevated
Thrombocytopenia +/- DIC
CSF cloudy with >1,000 WBC with many PMNs
Gram (-) intracellular diplococci
HPV - vaccine: composition ?
quadrivalent polysaccharides
6, 11
16, 18
HPV - vaccine: dosing ?
3 dose series
11-12 years
Newborn Screening ?
Blood samples collected by heelstick before hospital discharge
State-to-state variations in testing have diminished since 2010
Secretary Advisory Committee on Heritable Disorders in Newborns and Children set 30 core recommendations.