Step 2 Peds Flashcards
SGA infants are at risk for what complications?
SGA infants are at risk for hypoxia, perinatal asphyxia, meconium aspiration, hypothermia, hypoglycemia, hypocalcemia, and polycythemia. Polycythemia results from increased EPO secretion in response to fetal hypoxia.
PDT for laryngomalacia
collapse of supraglottic tissues on inspiration causes inspiratory stridor, peak at 4-8 months, dx laryngoscopy, management=reassurance, close f/u, likely GER tx, and supraglottoplasty in severe cases
Nontender abdominal mass associated with increased vanillylmandelic acid and homovanillic acid
neuroblastoma
Most common type of tracheoesophageal fistula. Classic presentation?
Esophageal atresia with distal TEF (85%). Unable to pass the NG tube.
Mild illness and/or low grade fever-OK for vaccines?
Yes
Current antibiotics-OK for vaccines?
Yes
Prematurity-OK for vaccines?
Yes
Tests to rule out abusive head trauma
optho exam, head CT + MRI
Neonate w meconium illeus might also have what disease?
Cystic fibrosis (Hirschsprung disease is associated w failure to pass meconium for 48 hours)
Billious emesis within hours of the first feeding
duodenal atresia
2 month old baby w non billious projectile emesis. Dx? Appropriate steps in management?
Pyloric stenosis. Hydrate, correct metabolic abnormalities, then pyloromyotomy
Most common primary immunodeficiency?
Selective IgA deficiency
Infant has high fever and rash onset as fever breaks
Febrile seizure due to roseola infantum
Boy w chronic respiratory infections. Nitroblue tetrazolium is negative. What is the immunodeficiency?
Chronic granulomatous disease
Child has eczema, thrombocytopenia, and high level of IgA. What is the immunodeficiency?
Wiskott-Aldrich Syndrome
4 month old with life threatening Pseudomonas infection. What is the immunodeficiency?
Bruton’s x-linked agammaglobulinemia
Acute phase treatment for Kawasaki disease?
High-dose ASA for inflammation and fever; IVIG to prevent coronary artery aneurysm
Treatment for mild and severe unconjugated hyperbillirubinemia?
mild: phototherapy
severe: exchange transfusion
Sudden onset of mental status change, emesis and liver dysfunction. Parents gave baby some med for fever yesterday. Dx?
Reye syndrome
Child lacks red eye reflex. Dx? increased risk of what cancer?
Retinoblastoma. Increased risk of osteosarcoma
Vaccinations at 6 months?
Hep B, DTaP, HiB, IPV, PCV, rotavirus.
What is the immunodeficiency?
Infection of small airways with epidemics in winter and spring
RSV bronchiolitis
Cause of neonatal RDS
surfactant deficiency
Colicy abdominal pain, billious vomting, sausage shaped mass in RUQ
Intussusception (also red currant jelly stools)
Vaccine schedule w mnemonic: Be / DR HIP / HHave2VeryManyPatients/DIM/Hurt Many Teens
Birth: Hep B
2, 4, 6: DtaP, Rotavirus, HiB, Hep B (2,6 only), IPV, PCV
12: HHave 2 Very Many Patients: HiB, Hep A, VZV, MMRV, PCV
4-6 yo: DTaP, IPV, MMRV
Teens: HPC, MCV, Tdap
Congenital heart disease that causes secondary hypertension. What would you find on physical exam?
Coarctation of the aorta. Decreased femoral pulses.
Tx for otitis media
amox
Most common pathogen that causes croup
parainfluenza virus type 1
Homeless child small for his age, peeling skin, swollen belly
Kwashiorkor (protein malnutrition)
Defect in x-linked syndrome with mental retardation, gout, self-mutilation and choreoathetosis
Lesch-Nyhan syndrome (purine salvage problem with HGPRTase deficiency)
Newborn with machine like murmur. What drug do you give?
PDA. Indomethacin closes it.
Newborn w posterior neck mass and swelling of hands. Dx?
Turner syndrome
Child w proximal muscle weakness, waddling gait and pronounced calf muscles.
Duchenne muscular dystrophy
First born female in breech position found to have aysymmetric skin folds on newborn exam. Dx? Tx?
Developmental dysplasia of the hip. If severe, consider a Pavlik harness to maintain abduction.
11 yo african american boy w sudden onset of limp
Slipped capital femoral epiphysis. AP and frog-leg lateral radiographs
An active 13 yo w anterior knee pain. Dx?
Osgood-Schlatter Disease
Out of the R–>L heart defects, which one shows up within the first few hours of life w severe cyanosis?
Transposition of the great vessels (2 arteries are switched)
Which one? Neisseria vs. chlamydia isolated on vaginal cx of child definitively indicates sexual abuse?
Nisseria. Chalymdia can be acquired from mother during delivery and can persist for up to 3 years
What are the 3 L–>R shunts?
3D’s and C:
VSD, ASD, PDA + Coarctation
Which heart defect has a fixed, widely split S2?
ASD
In Eisenmenger syndrome, a left to right shunt leads to what?
Pulmonary hypertension and shunt reversal
ASD and endocardial cushion defects are associated with what disorder?
Down syndrome
PDA is associated with what disorder?
Congenital rubella
Coarctation of the aorta is associated with what disorder?
Turner syndrome (many pts also have a bicuspid aortic valve
Coronary artery aneurysm is associated with what disorder?
Kawasaki syndrome
Congenital heart block is associated with what disorder?
Neonatal lupus
Supraclavicular aortic stenosis is associated with what disorder?
Williams syndrome. Supraclavicular aortic stenosis is the least common form of left ventricular outflow tract obstruction. Williams syndrome is also characterized by unusual facial features, intellectual disabiity and hypercalcemia
Conotruncal abnormalities are associated with what disorder?
Tetrakigt if Fakkit (overiding aorta), truncus arteriosus, DiGeorge syndrome, velocardiofacial syndrome
Ebstein anomaly is associated with what disorder?
Lithium use during pregnancy
Heart failure is associated with what disorder?
neonatal thyrotoxicosis
Asymmetric septal hypertrophy and transposition of the great vessels is associated with what disorder?
Maternal diabetes
Auscultation findings of ASD
Wide fixed split S2, systolic ejection murmur at LUSB, (increased flow across pulmonary valve). Mid-diastolic rumble at LLSB.
Auscultation findings of VSD
Harsh holosystolic murmur at LLSB. Narrow S2 with increased pulmonic component. Mid-diastolic apical rumble (due to increased flow across the mitral valve).
ECG findings of ASD
RVH. Right atrial enlargement, PR prolongation
DDX for infants (0-4 weeks) presenting w shock-like symptoms?
- sepsis
- inborn errors of metabolism
- ductal-dependent congenital heart disease, usually left-sided lesions (as the duct is closing)
- CAH
DiGeorge Syndrome: CATCH 22
Cardiac Abnormalities (transposition) Abnl face Thymic aplasia Cleft palate Hypocalcemia 22q11 deletion
What is the most common cyanotic heart disease of newborns?
Transposition of the great vessels?
What is the most common cyanotic heart disease of children?
Tetraology of Fallot 1. Overriding Aorta 2. VSD 3. RV outflow tract obsturction 4. RVH Boot shaped heart
Intial treatment for both transposition of great vessels and tetraology of Fallot?
PGE to keep the PDA open, but then surgical correction
What are common signs of autism
No babbling by 12 motnsh, no two-word phrases by 24 months, impaired social interaction, restricted interests and insistence on routine.
Age for delayed puberty in girls? boys?
girls=13, boys =14
Characteristics of Edwards syndrome
Rocker bottom feed, low-set ears, micrognathia, clenched hands, prominent occiput, assc’d w congenital heart disease and horseshoe kidneys. death by 1 yo
Characteristics of Patau syndrome
Micropthalamia, microcephalmia, cleft lip/palate, holoprosencephaly, punched out scalp lesions, polydactyly, omphalocele, assc’d w congenital heart disease. death witnin y yo
Tall stature, gynecomastia, female hair distribution. Dx?
Kleinfelter syndrome (XXY), treat w testosterone
PKU PDT
P/w intellectual disabilities, fair hair and skin, eczema, blond hair, blue eyes, and musty urine. Assc’d increased risk of heart disease. autosomal recessive
Dx w newborn screen
Tx decreased phenylalanine and increased tyrosine
Fragile X P D T
P: large jaw, testes,and ears, autistic behavior
D: genetic: FMR1 gene
Tx none
Fabry Disease PD? deficiency of? inheritance?
P : first sign is severe neuropathic limb pain, also presents with joint swelling. Other findings include renal failure, and an increased risk of stroke and MI (thromboembolic events). Skin infolgement takes the form of angiokeratomas and telangiectasias
Dx: XLR deficiency of alpha-glactosidase A that leads to accumulation of ceramide trihexoside in heart, brain, and kidneys
Krabbe Disease PD? deficiency of? inheritance?
P: Charaterized by progressive CNS degeneration, optic atrophy, spasticity, and death within first 3 years of life. Autosomal recessive
D: visulazie globoid cells. Absence of glactosylceramide and galactoside (due to galactosylceramidase deficiency) leading to acculumation of falactocerebroside in the brain
Gaucher Dz PD? deficiency of? inheritance?
P: Pancydopenia, early, rapid neurologic decline (infantile form) or adult form compatible with nl life span. Organomegaly of liver, spleen.
D: Autosomal recessive. Caused by a deficiency of glucocerebrosidase that leads to the accumulation of glucocerebroside in brain, liver, spleen, and bone marrow. Gaucher cells have a characteristic crinkled paper-like appearance w enlarged cytolasm.
Nieman-Pick Disease PD? deficiency of? inheritance?
P: cherry red spot and HSM. Patients w type A die by 3 yo
D: autosomal recessive. Deficiency of sphingomyelinase that leads to buildup of sphingomyelin cholesterol in reticuloendothelial and parenchymal cells and tissues
Mne: No man PICKs (NiemanPick) with his sphinger.
Tay-Sachs PD deficiency of? inheritance?
P: Infants may be nl until 3-6 months then weakness appears and development slows and regresses. An exaggerated statle respnse may be seen. P/w cherry red spot BUT NO HSM.
D: Autosomal recessive. 1/30 Ashkenazi jews are carriers.
Mne; Tax-Sax lacks heXosaminidase (leading to GM ganglioside accumulation
Metachromatic leukodystrophy PD deficiency of? inheritance?
P: deymelination, ataxia, dementia
D: autosomal recessive. Deficiency of arylsulfatase A that leads to acculumation of sulfatide in brain, kidney, liver and peripheral nerves
Hurler syndrome. deficiency of? inheritance?
Deficiency of alpha-L-iduronidase.
Pts present w corneal clouding, intellectual disabilities and gargoylism. Autosomal recessive
Hunter syndrome. deficiency of? inheritance?
Deficiency of iduronate sulfatase. A mild form of hurler syndrome w NO CORNEAL CLOUDING. and mild intelectual disabilities. XLR.
Mne: Hunters need to see (no corneal clouding) to aim for the X
Nearly all cases of meconium ileus are due to what genetic disease?
cystic fibrosis
Most cases of pyloric stenosis occur between what months of life?
3 weeks-3 months
Meckels rule of 2s? and PDT
Occurs in 2% of population, 2% symptomatic by age 2, 2x more common in boys, contains 2 types of tissue (gastric and pancreatic), 2 inches long, found within 2 feet of the ileocecal valve.
P: painless rectal bleeding, most cases discovered incidentally
D: technetium 99 pertechnetate scan
T: surgical excision
Most common cause of bronchiolitis?
RSV
Most common cause of croup?
Parainfluenza
Kawasaki disease symptoms
CRASH and BURN
C: Conjunctivitis, sparing limbic area
R: Rash, primarily truncal, polymorphous, erythematous
A: Adeonapathy (unilateral), generally painful
S: strawberry tongue
H: hands and feed (red, swollen, flaky skin)
BURN: fever>40C/104F for 5 or more days
Common bugs of meningitis for newborns? infants/children? adolescents?
newborns: GBS, E.Coli, Listeria
infants/children: Strep pneumo, Nisseria, Haemophilus
adolescents: Nisseria, Strep pneumo
What are the risks for giving neonates CTX?
biliary sludging + kernicterus
Most common childhood malignancy?
ALL, followed by CNS tumors and lymphoma
What are the lab characteristics of tumor lysis syndrome?
hyperkalemia, hyperphosphatemia, hyperuricemia, and hypocalcemia (bc calcium is bound by phosphate that is released from the neoplastic cells). Corticosteroids may precipitate tumor lysis syndrome
DDX of limp in Pediatric pt? STARTSS HOTT
Septic joint Trauma Avascular necrosis Rheumatoid arthritis/JIA Tuberculosis Sickle cell disease Slipped caiptal femoral epiphysis
Henoch-Schonlein purpura
Osteomyelitis
Tumor
Toxic synovitis
Selective IgA Deficiency. PDT.
The most common primary immunodeficiency syndrome.
Clinical features
Often asymptomatic
May manifest with sinusitis or respiratory infections (S. pneumoniae, H. influenzae), chronic diarrhea (Giardia), steatorrhea
Associated with gluten-sensitive enteropathy, inflammatory bowel disease
Diagnosis: serum IgA level < 7 mg/dL, with normal IgG and IgM levels
T:ABX
Sketch:
Great Bruton Play
