9 Flashcards
most important initial test to do when thinking Guillain-Barre
spirometry to assess respiratory function
spinal MRI is nonspecific
stridor in laryngomalacia vs vascular ring
stridor in LGM is inspiratory and improves with prone positioning, in vascular rings it is biphasic (insp. and exp.) and it improves with neck extension
hx of easy bruising, bleeding after dental procedure, recurrent joint swelling, and family history of similar, consider?
mechanism?
hemophilia leading to hemophilic arthropathy
iron/hemosiderin deposition in the joints leading to synovitis and fibrosis in the joint
neonate presenting with poor sucking, fatigue, rigidity, spasms after lack of pre/postnatal care and swollen umbilical cord at delivery, think ?
tetanus
infant presenting with irritability, high-pitched cry, poor sleeping, tremors, seizures, sweating, sneezing, tachypnea, poor feeding, vomiting, diarrhea, think mom abused ? while pregnant
heroin
neonatal abstinence syndrome is caused by infant withdrawal to opiates, presents in 1st few days of life
hypotonia, areflexia, hepatosplenomegaly, and cherry red macula
Neimann-Pick disease (sphingomyelinase deficiency)
ddx from Tay-Sachs (B-hexosaminidase A deficiency); which will not present with areflexia or hepatosplenomegaly
Down syndrome pt with upper motor neuron findings, think?
atlantoaxial instability
SGA infants are at risk for
hypoxia–>polycythemia (from increased EPO), hypoglycemia, hypocalcemia, hypothermia
if transient proteinuria (i.e. 2+ on dipstick is found)
reevaluate with 2x repeat urine dipstick to rule out persistent proteinuria
Osgood-Schlatter is caused by ?
findings?
a traction apophysitis of the tibial tubercle
anterior tissue swelling, lifting of tubercle from shaft and irreg/fragment of tubercle
life-threatening complication days-months after cardiac surgery
postpericardiotomy syndrome: pericardial effusion
distant heart sounds, hypotension, distended jugular veins
-requires drainage
triad of thrombocytopenia (sm plts too), eczema, and recurrent infection
Wiskott-Aldrich
left-axis deviation on neonatal EKG and decreased pulmonary markings
NEVER normal, hypoplasia of RV and pulmonary outflow tract
i.e. tricuspid valve atresia
if thinking malrotation with midgut volvulus after abdominal Xray, what is the next diagnostic step?
Upper GI series (barium swallow)
finding of Ligament of Treitz on the right side of the abdomen reflects malrotation while contrast in a corkscrew pattern indicates volvulus
fix with the Ladd procedure
what conditions are contrast enemas used for
Hirschsprung and meconium ileus
most common complication of patients with sickle cell TRAIT
Hbg % ?
painless hematureia
HbA: 50-60%
HbS: 35-45%
HbF: less than 2%
nonimmune, asymptomatic healthy pt older than 1 exposed to varicella, what to do?
receive varicella vaccine (post-exposure prophylaxis)
kids younger than 1 year are more likely to have ? hematomas; ? hematomas are more common in older children
subdural
epidural
seizure are more common with epidural or subdural hemorrhage?
subdural
which has a higher mortality; epidural or subdural hemorrhage?
epidural, but subdural has more significant long-term morbidity; brain parenchyma is more often involved.
approximately ? of CT-identified subdural hemorrhages have an associated skull fracture; almost all are ? in origin, and approximately ? are bilateral
1/3
venous
3/4
symptom timeline of acute, subacute, or chronic subdural hemorrhage
acute: 1st 48 hrs after injury
subacute: 3-21 days
chronic: after 21 days
Chronic subdural hematomas are more common in older children or infants?
symptoms may include ?
imaging?
older children
chronic emesis, seizures, hypertonicity, irritability, personality changes, inattention, poor weight gain, fever, and anemia
MRI over CT
? of epidural hemorrhages are associated with skull fracture
most adult epidural hemorrhages are arterial in origin, but in kids about ?originate from venous injuries
Most are unilateral or bilateral?
2/3
50%
unilateral,located in the temporoparietal region
symptoms of ICP in infants with open sutures
may be nonspecific and include lethargy, vomiting, separated sutures, and a bulging fontanelle
treatment of ICP in epidural vs subdural hemorrhage
Epidural hematomas are frequently rapidly progressive and may require urgent surgical evacuation with identification of the bleeding source
Subdural hemorrhage usually does not require urgent evacuation but may require evacuation at a later date
how to treat symptomatic hypoglycemic infants
IV bolus of dextrose, the D-isomer of glucose (200 mg/kg), is given over 5 minutes (2 mL/kg of 10%dextrose in water [D10])
followed by the continuous administration of parenteral dextrose infusion at a rate of 6 to 8 mg/kg of dextrose per minute
The maximum dextrose concentrations for fluid administered through a peripheral IV catheter or a low lying umbilical venous catheter is ?%, and through a central venous catheter (including a centrally positioned umbilical venous catheter) is ?%
12.5%
25%
febrile seizure
A seizure occurring in the absence of a CNS infection with an elevated temperature in a child between the ages of 6 months and 6 years
simple vs complex febrile seizure
simple: less than 15 min, short post-ictal state, no recurrence in 24 hrs, 80% of febrile seizures
complex: either focal, lateralizing signs, prolonged, or recurrent, 20%
when to get an LP with febrile seizure
- neurologic examination is abnormal after the seizure, the seizure occurred several days into the illness, or if the child is unable to provide adequate feedback during a neck examination, in child less than 9mo with fever and seizure particularly those infants with deficient or unknown immunizations
- if less than 1 yr consider CT, EEG +/- LP
Seizures lasting longer than 5 minutes may be interrupted with ?
if unresponsive to those, try ?
lorazepam or diazepam
fosphenytoin
Children younger than 12 months at the time of their first seizure have a ?% chance of having another febrile seizure; older children have a ?% chance of recurrence
50% to 65%
20% to 30%
kids at highest risk for developing epilepsy after febrile seizure
preexisting neurologic problems and have complex febrile seizures; these children have 30 to 50 times the baseline risk of developing epilepsy.
ddx febrile seizure
meningitis, bacterial enteritis, subdural hematoma/head trauma, stroke from SCD, CP
Self-limited scaly, erythematous, and/or crusty eruption limited to areas of the skin with a high concentration of sebaceous glands (cradle cap
seborrheic dermatitis
General term for a skin condition consisting of acutely inflamed papules and plaques, frequently associated with serous discharge and pruritus; eczematous eruptions include atopic, seborrheic, and contact dermatitides
eczema
most cases of AD are precipitated by ? and not ?
environmental stress on genetically compromised skin and not by interaction with allergens
ddx atopic dermatitis
seborrheic dermatitis (cradle cap), scabies, irritant dermatitis (perioral fruit juice dermatitis), allergic contact dermatitis (poison ivy, oak, sumac (Toxicodendron)), and eczematoid dermatitis (infectious lesion near a draining ear)
rare ddx of dermatitis
ichthyosis, severe combined immune deficiency (SCID), Wiskott-Aldrich syndrome (eczema, thrombocytopenia, and immunodeficiency), zinc deficiency, and drug reactions.
good rash questions
how long? previous episodes? pruritic? itching at night? irritable? worse with exposure to cold, wool, sweat, stress? family members with eczema, asthma, allergies? +fever or other signs of infection?
good products for atopic dermatitis
nonsoap cleansers (Cetaphil), Lubricants (Eucerin), products with urea (Nutraplus) or lactic acid (Lac-Hydrin)
only FDA-approved topical corticosteroid creams for infants as young as 3 months
Fluticasone propionate 0.5% cream (Cutivate) and desonide 0.05% gel (Desonate)
Fluorinated corticosteroids are generally avoided on the ? because they may depigment and thin the skin
face, genitalia, and the intertriginous area
nonsteroidal, immunomodulator topicals FDA approved for the treatment of AD in children 2 years and older (if not adequately responsive to, or intolerant of, conventional therapy)
Tacrolimus 0.03% (Protopic) and pimecrolimus 1% (Elidel)
**black box warning for association with LN/skin malignancies
reduce itching in AD with
hydroxyzine, diphenhydramine (night)
loratadine (Claritin) and cetirizine (Zyrtec)- less sedating
Doxepin (Sinequan): TCA and antihist. effects
don’t use topical antihists: (Caladryl)
to reduce secondary bac infection with AD
mupirocin (Bactroban) -for staph aureus
“id” reaction
widespread pruritic papules in nonexposed areas
In infancy, the itchy eruption of AD is found ?; by childhood, the rash is noted ?
on the face and cheeks
in flexural areas
how to tx seborrheic dermatitis
baby shampoo
antidandruff shampoo containing antifungal medication (Nizoral) or selenium
ketoconazole
low-to-medium-potency topical corticosteroids
oral lesions, weight loss, and lymphadenopathy think?
immunodeficiency
Recommended assay to diagnose HIV infection in children younger than 18 months
HIV DNA PCR (2x)
instead of ELISA (Ab test) because of placental transfer of maternal antibodies
used to assess response to antiretroviral therapy (ART) in patients diagnosed with HIV
HIV RNA PCR
Detects HIV immunoglobulin G (IgG); initially detectable 2 weeks to 6 months after exposure
ELISA
CD4+ T helper cells bind to antigens presented by the ? on the surface of antigen presenting cells (APCs), prompting ?
Class II MHC molecules
chemokine release and immune activation; rendered dysfunctional in HIV infection.
causes of Primary (syndromic) immunodeficiency
SCID, thymic dysgenesis in DiGeorge syndrome, phagocytic cell deficiency (chronic granulomatous disease due to impaired respiratory burst), complement deficiency (autoimmune disease or serious bacterial infection due to C2 deficiency), and neutrophil dysfunction (autosomal-recessive leukocyte adhesion deficiency)
treatment of primary immunodeficiency
compensating for the defective response (IV immune globulin in humoral defects) or, in severe cases, reconstitution of the immune system via bone marrow stem cell transplant
causes of secondary immunodeficiency
HIV infection, diabetes mellitus, sickle cell disease, malnutrition, hepatic disease, autoimmune disease, aging, and stress
If HIV+ mother received ART during pregnancy, how to treat infant?
if HIV+ did not receive ART?
- 6 weeks of ART in the form of zidovudine starting in the first few hours of life
- three doses of nevirapine added to standard zidovudine therapy during the first week of life (reduced transmission rate to 2.2%)
If an infant is diagnosed by two positive DNA PCR tests ?
zidovudine monotherapy should be discontinued, and the child referred to a pediatric HIV specialist for combination ART and monitoring
PCP prophylaxis in the form of TMP-SMX at 6 wks
adverse effects for all ARTs
common: headache, emesis, abdominal pain, and diarrhea
- Osteopenia and drug rash
- anemia, neutropenia, elevated transaminases, hyperglycemia, and hyperlipidemia
current pediatric ART recommendation consists of three drugs:
two NRTIs and either a protease inhibitor or a NNRTI
Immunizations for HIV+ kids
should be kept current EXCEPT live vaccines such as measles, mumps, and rubella (MMR) and varicella for symptomatic HIV-infected children with a CD4 count less than 15%.
if high suspicion of HIV and initial ELISA is negative, consider what next test?
HIV RNA PCR to detect replicating virus
CD4 count is premature
leukocyte adhesion deficiency (LAD)
inheritable disorder of leukocyte chemotaxis and adherence characterized by recurring sinopulmonary, oropharyngeal, and cutaneous infections with delayed wound healing
Neutrophilia is common with (WBC) counts typically more than 50,000 cells/mm3
Severe, life-threatening infection is possible with Staphylococcus species, Enterobacteriaceae, and Candida species
precocious puberty, elevated LH, and advanced bone age, consider ?
what diagnostic step?
Gonadotropin dependent precocious puberty (GDPP)
brain MRI with contrast
pts with HSP are at risk for what GI condition?
intussusception
absence seizures are associated with ?
ADHD
most common cause of bacterial pneumonia in young child with CF
S. aureus
Pseudomonas more common in adults (older than 20)
difficulty moving R arm and R leg after found unresponsive, think ?
Todd paralysis (after seizure)
aplastic anemia vs aplastic crisis
aplastic anemia shows pancytopenia due to BM failure
aplastic crisis shows severe anemia in SCD pts; low Hgb and retic (less than 1%), lack of hepsplenmeg, Parvovirus B19 is common trigger
pts with Tourette syndrome have an increased risk of developing what conditions
ADHD, OCD (other psych conditions as well)
how to tx Lyme disease in kid less than 8
amoxicillin (not doxy)
